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ABSTRACTS
Year : 2016  |  Volume : 11  |  Issue : 5  |  Page : 20-108

IRACON 2016: Poster Presentations


Date of Web Publication27-Oct-2016

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How to cite this article:
. IRACON 2016: Poster Presentations. Indian J Rheumatol 2016;11, Suppl S1:20-108

How to cite this URL:
. IRACON 2016: Poster Presentations. Indian J Rheumatol [serial online] 2016 [cited 2017 Jul 20];11, Suppl S1:20-108. Available from: http://www.indianjrheumatol.com/text.asp?2016/11/5/20/193141


  P1 Category: Spondyloarthropathy Top


Reduction of nitric oxide bioavailability in ankylosing spondylitis patients suffering from subclinical endothelial dysfunction

Ayindrila Saha, Sumantro Mondal, Alakendu Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education & Research, Kolkata, India

Background : Ankylosing spondylitis (AS) is characterised by inflammatory back pain with evidence of sacroiliitis in imaging. Cardiovascular complication is observed in AS and it is associated with endothelial dysfunction (ED). However the exact pathway responsible for ED is not clearly known. In this study we detected ED and subclinical atherosclerosis in AS patients & control population, along with measurement of serum nitric oxide level.

Methods: 40 AS patients (age--30.16 ± 6.14 yrs, 30 males, and 10 females) and 30 healthy controls (28.92 ± 4.724, 21 male, 9 females) were enrolled. Bath Ankylosing Spondylitis Functional Index (BASFI) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) were evaluated. Flow-mediated dilatation (FMD) of the brachial artery and carotid intima-media thickness (cIMT) were measured sonographically as per guideline. The serum levels of nitric oxide were determined by colorimetric assay and serum lipid profile and acute-phase reactants were also assessed.

Results: ED-FMD is significantly lower in AS patients compared to control groups (10.10 ± 1.116 vs 15.05 ± 0.0807, p-value = 0.0013). Likewise nitric oxide concentrations are much lower in AS patients than control group (3.86 ± 0.495 vs 6.088 ± 0.862, p-value = 0.0246). There is no significant intergroup c-IMT difference.

Conclusion : Endothelial dysfunction plays a major role in altered vascular patho-physiologic dynamics in AS, which may be related to reduced serum concentration of nitric oxide.


  P2 Category: Spondyloarthropathy Top


Spironolactone Increases Nitric Oxide Bioactivity, Improves Endothelial function and Suppresses Inflammatory Disease Activity in Patients with Ankylosing Spondylitis

Ashit Syngle1 , Inderjeet Verma2 , Pawan Krishan2 ;1 Director-Cardio Rheuma & Healing Touch City Clinic, Chandigarh and Rheumatologist Fortis Multi Specialty Hospital, Mohali,2 Department of Pharmaceutical Sciences and Drug Research, Punjabi University, Patiala, India

Background: Nitric oxide (NO) plays a central role in the regulation of blood vessel tone and inflammation. Therefore, NO inhibitors represent important therapeutic advance in the management of inflammatory diseases. Treatment with spironolactone (SPIR), increases NO bioactivity and improves endothelial function in patients with heart failure.In the present study, we investigated the serum nitrite levels in patients with ankylosing spondylitis (AS) patients after treatment with spironolactone.

Methods: 50 patients (25 in SPIR (2 mg/kg/day) and 25 in placebo arm) meeting the modified New York criteria for AS, with active disease despite treatment with conventional DMARDs were recruited. Therapy results were evaluated by serum nitrite level and flow mediated dilatation (FMD) was assessed by AngioDefender. Inflammatory measures ESR, CRP, BASDAI, BASFI and pro-inflammatory cytokines (TNF-α, IL-6 and IL-1) were also assessed at baseline and after 24 weeks.

Results: After 24 weeks, a significant improvement in serum nitrite was observed in spironolactone group (4.92 ± 0.70 to 4.08± 0.48, p=0.01) compared with placebo (5.02 ± 0.75 to 4.93 ± 0.64, p=0.18). After treatment with SPIR the mean value of the FMD improved from 5.83 to 7.82 (p<0·001) and with placebo from 5.87 to 6.11 (p=0·12). At 24weeks, TNF-α, IL-6 and IL-1 improved significantly in SPIR compared with placebo. At 24 weeks; ESR, CRP, BASDAI and BASFI significantly (p<0·05) improved in SPIR group compared with placebo.

Conclusion: These results suggest that SPIR significantly reduced serum nitrite concentration and consequent improvement in inflammatory disease activity, endothelial dysfunction and provides a promising strategy to manage AS and its complications.


  P3 Category: Pediatric Rheumatology Top


Foot Involvement in Enthesitis-Related Arthritis category of Juvenile Idiopathic Arthritis- Clinical, Radiological and Functional Assessment

Sanat Phatak, Namita Mohindra, Abhishek Zanwar, Amita Aggarwal; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

Introduction: Though foot involvement is common in JIA, it is often neglected by patients and physicians alike. The involvement could occur due to arthritis, enthesitis or tenosynovitis. Tarsitis is thought to be a harbinger for spondyloarthropathy. Thus this study was done to assess clinical and radiological involvement of feet as well as its impact on function in children with JIA-ERA.

Methods: We enrolled consecutive patients newly diagnosed with JIA ERA of age less then 18 years and disease duration less than 5 years seen during 2015. All patients underwent clinical examination of the feet and answered the Juvenile arthritis foot index (JAFI) questionnaire to assess functional impact. Radiological assessment included plain X-rays of the feet, US of the joints and entheses and MRI scan of one foot. HLAB27 was done by PCR.

Results:
Fifty-five patients, with a median age of 14 years and duration of disease 1.9 years were included in the study. Eighty percent (37 of 46) were HLAB27 positive and 18 (32.7%) had radiographic sacroiliitis. Forty six (83.6%) patients had a history of foot pain while 36 had abnormal clinical exam: 15 had ankle, 8 had subtalar 24 had midfoot and 10 had forefoot joint involvement. Tendoachillesenthesitis was present in 21 whereas plantar fasciitis was seen in 7 patients. The median JAFI score was 4 (0-11) and the score correlated with history of foot pain (r=0.66 p less than 0.01) and foot swelling (r 0.58, p less than 0.01). On plain X-ray (N 40) 3 had midfoot joint space reduction and 3 had midfoot joint fusion. On US (N 55), 16 had ankle, 8 had subtalar and 19 patients had midfoot arthritis with the talonavicular (TN) joint being the most commonly involved. 27 had US active tendoachillesenthesitis and 11 had plantar fasciitis. On MRI (N=50) tarsitis was seen in 27 patients and TN was the most commonly involved joint. Bone marrow edema was seen in 33 patients with the calcaneum being the most commonly affected bone. Midfootenthesitis was found in 14 patients and tenosynovitis in 17 patients. Clinical and US had 82% concordance at midfoot and 90% at tibiotalar joint. MRI had lower concordance rates (74% with clinical exam and 72% with ultrasound at midfoot) as it picked up additional findings in 12 patients.

Conclusion: Foot joints and entheses are involved in a substantial proportion of patients with JIA ERA patients and lead to functional disability. Early recognition and treatment may decrease damage.


  P4 Category: Spondyloarthropathy Top


Serum levels of IL-17 and IL-23 as predictors of chronicity in Reactive arthritis

Bhuvanesh Mahendran, Rajeswari Sankaralingam, Balaji Chilkuri, Saranya Chinnadurai, Natesan Tiruchengode Tamilselvam; Institute of Rheumatology, Madras Medical College, Chennai, India

Background: An estimated one third patients with Reactive arthritis (ReA) develop chronic sequelae (lasting more than 6 months) or chronic spondyloarthritis (SpA). Predicting which subsets of patients develop chronic arthritis has remained an enigma.

Objective:
To investigate the role of IL-17 and IL-23 as predictors of chronicity in ReA.

Methods:
25 patients with reactive arthritis were included in this study. ESR, CRP, serum IL-17 and IL-23 levels were assessed at baseline and at end of 3 months of disease duration. Patients were assessed at baseline, 2 and 3 months of disease duration. Non-remission of arthritis was taken as persistence of arthritis in at least one joint at the end of 3 months of disease duration. 30 healthy age and sex matched controls were also included in this study.

Results:
Demographic parameters including age and sex were comparable between the patient and control group. Patients with ReA had higher levels of serum IL-17 (mean 18.1pg/ml) and IL-23 (mean 358.2pg/ml) when compared with healthy controls (mean 4.3pg/ml, p<0.001 and mean 157.4pg/ml, p<0.001). Out of 25 patients with ReA, 8 patients (32%) had persistence of arthritis at 3 months. Serum levels of IL-17 and IL-23 were significantly higher at baseline in patients who had persistent symptoms (mean 39.1pg/ml, p<0.001; mean 682.5pg/ml, p<0.001) compared to those who had complete remission (mean 8.22pg/ml; mean 205.7pg/ml). In patients who had chronic arthritis there was persistent elevation of both IL-17 and IL-23 levels at 3 months. There was no correlation between inflammatory markers ESR, CRP and serum IL-17 and IL-23 levels.

Conclusion:
Markedly elevated serum levels of IL-17 and IL-23 at baseline can predict the development of chronicity in reactive arthritis and may call for aggressive initial therapy in this subset of patients.




  P5 Category: Spondyloarthropathy Top


Stem cell therapy in Psoriatic Arthritis: Exploring Endothelial Progenitor Cells (EPCs)

AshitSyngle1 , NidhiGarg2 , PawanKrishan3 ; Fortis Multi Speciality Hospital, Mohali, India, Cardio Rheuma & Healing Touch City Clinic, Chandigarh & Consultant Rheumatologist- Fortis Multi Speciality Hospital, Mohali, Department of Pharmaceutical Sciences & Drug Research, Punjabi University

Background: Cardiovascular disease is the leading cause of morbidity and mortality in Psoriatic Arthritis (PsA).Endothelial Progenitor Cells ( EPCs) have a vasculoprotective effect but are depleted and contribute to increased cardiovascular (CV) risk in PsA. Statins have a protective effect in coronary artery disease but this has not yet been investigated in PsA. We aimed to investigate the effect of rosuvastatin on EPCs in PsA.

Methods:
36 PsA patients were randomized to receive 24 weeks of treatment with rosuvastatin (10mg/day, n=18) and placebo (n=18) as an adjunct to existing stable antirheumatic drugs. EPCs (CD34+/CD133+) quantified by Flow Cytometry. Inflammatory measures (DAS28, DAPSA, CRP and ESR) and pro-inflammatory cytokines (TNF-α, IL-6 and IL-1) measured at baseline and after treatment. Lipids were estimated at baseline and after treatment.

Results: At baseline, inflammatory measures and pro-inflammatory cytokines were elevated and EPCs depleted among both groups. At baseline, EPCs inversely correlated with DAS28 and CRP in both groups and EPCs increased significantly (p<0.01) after treatment with rosuvastatin. Rosuvastatin exerted positive effect on lipid spectrum. DAS28, DAPSA, ESR, CRP, TNF-α and IL-6 improved significantly in rosuvastatin group. Significant negative correlation was observed between EPCs and DAS28, CRP and IL-6 after treatment with rosuvastatin.

Conclusion:
Rosuvastatin improves inflammation and EPC biology in PsA possibly through its anti-inflammatory effect. Rosuvastatin lowers the cytokines, especially IL-6 and TNF-α, which downregulates CRP production. This suggests that cholesterol lowering per se has anti-inflammatory effects and improves vascular function in PsA, indicating favourable effects of rosuvastatin on the cardiovascular system in PsA.


  P6 Category: Spondyloarthropathy Top


Co-relation of trough drug levels and disease activity measures in infliximab treated Spondyloarthritis patients: A cross sectional analytical study

AbhishekPatil, BhupendraVaishnav, ReetikaDawar1 , SundeepUpadhyaya, Sirinder J Gupta, RohiniHanda;1 Department of Rheumatology, Microbiology Indraprastha Apollo hospitals, Sarita vihar, Delhi

Background/Purpose: Infliximab (IFX) is commonly utilized in treatment of spondyloarthritis (SpA) patients. The study was undertaken to evaluate whether the trough drug levels co-relate with disease activity in IFX treated SpA patients

Methods: Fifteenpatients of axial or peripheral SpA between 18-70 years who had received 3 or more infusions of IFX (3-5 mg/kg) over a period of 6 months or more were recruited into study. Their disease activity was quantitated using ASDAS- ESR and ASDAS- CRP scores. Trough IFX levels (between 2- 3 months after previous infusion) were determined by using Matriks biotech Shikari Q-INFLIXI ELISA kits by solid phase enzyme-linked immunosorbent assay (ELISA) based on the sandwich principle. Co-relation between ASDAS scores and trough IFX were analyzed by Pearson's correlation assay.

Result: The mean trough IFX concentration was 1.49 ±.96 μg/ml and corresponding mean ASDAS - ESR/ CRP scores were 2.76 ± 2.12 and 2.59 ± 2.26 respectively. There was a significantnegative co-relation between trough IFX levels and ASDAS-ESR (r= -0.6781, p<0. 01) and ASDAS CRP scores (r= -0.6245, p<0.05). One in five patients with serum trough IFX ≥1.5 μg/ml had high disease activity, whereas 8/10 patients with drug levels <1.5 μg/ml had high disease activity measured by either ASDAS-ESR/CRP.

Conclusion- Even at lower doses IFX therapy (3-5 mg/kg), Serum trough IFXlevels have a significant negative co-relation with disease activity and hence can be used to monitor and calibrate drug dose in SpA patients.




  P7 Category: Spondyloarthropathy Top


Hearing Loss InAnkylosing Spondylitis

SajalAjmani1 , Able Lawrence2 , RakeshSrivasatva3 , AmitKesari4 ;1 Department of Clinical Immunology and2, 3 Department of Neuro-otology, SGPGIMS, Lucknow, India

Background : Several studies have described sensorineural deafness in ankylosing spondylitis (AS) patients, while conductive hearing loss has been reported to be rare. We have studied the prevalence of hearing loss (HL) in AS.

Methods : We studied 100 patients of AS fulfilling modified New York criteria and 30 controls. HL was considered to be present when the audiometric tests disclosed pure-tone thresholds greater than 20 dB (decibel) in at least 2 frequencies. Mild, moderate, moderately-severe, severe and profound HL were defined as HL range 25-40, 40-55, 55-70, 70-90, >90 dB respectively. Clinical details such as age, disease severity parameters and cumulative NSAID dose were noted. All variables are expressed as median (25th - 75th Interquartile range)

Results : Ninety-six of the 100 patients were male patients. The median age was 32 (23-42) years and median duration of illness was 6 (2-12) years. Median BASDAI, BASFI, BASMI and cumulative NSAID dose were 3(1.7-4.9), 2(1-4.15), 3.6(1.6-5.5) and 726 (210-1825) respectively. Of the 48 patients with HL, 28 patients had bilateral HL. Twenty-nine patients had pure conductive HL while 16 had mixed HL and 3 had pure sensory neural HL. Hearing loss was mild in 38 patients, while 10 had moderate to severe HL. Presence of HL was associated with higher age (p=<0.05). Conductive HL, when present, was at low frequency (0.25, 0.5, 1 kHz) in 70% cases. Sensorineural HL, when present, was at high frequency (4, 8 kHz) in 75% cases. There was no association of presence of HL with disease parameters such as BASMI, BASDI,BASFI or cumulative NSAID dose. Among the controls 5 (16%) had HL, all of which were of mild to moderate conductive type.

Conclusion : Hearing loss is common among patients of AS (Table-1). Conductive HL is more common than sensorineural HL. It usually mild and occurs at low frequency (Figure-1).



AS patients had a wide Air-Bone gap (arrow) suggestive of conductive hearing loss at low frequency


  P8 Category: Spondyloarthropathy Top


TNFα blockers followed by cDMARDs - cost effective option for treatment of spondyloarthritis



Aswin M Nair1 , Sandhya P1 , BijeshYadav2 , Debashish Danda1 ; Department of Rheumatology & Biostatistics, Christian Medical College, Vellore, India

Background:
Costs deters continuous use of tumor necrosis factor α (TNF α) blockers in India. The objective of this study was to evaluate outcome and expenditure incurred in SpA patients beyond a year of follow up after receiving 4 doses of Infliximab (IFX) on a background therapy of methotrexate (MTX) and sulfasalazine (SSZ).

Methods: Patients with SpA satisfying the Assessments in Ankylosing Spondylitis International Society criteria (ASAS) between 2008 - 2014 were screened. Those who completed at least one year of follow up after receiving 4 doses of IFX (5 mg/kg at 0, 2, 6 and 14 weeks) on a background therapy of MTX (10-25 mg/week) and SSZ (2-3 g/day) were enrolled after obtaining informed consent. Primary outcome assessed was duration to disease flare. Changes in acute phase reactants (ESR, CRP), patient reported outcomes (BASDAI, BASFI) and cost effectiveness were also assessed.

Results: Forty-five patients were enrolled. Mean (SD) duration of follow up after the 4th IFX dose was 28.9(18.7) months. Disease flare occurred in 33.3% (15/45) after a mean duration of 14.3(10.8) months as compared to 4-6 months described in literature on discontinuing anti-TNF therapy. Reduction in ESR, CRP, BASDAI and BASFI continued to be statistically significant at follow up as compared to baseline. As compared to recommended continuous IFX therapy, treatment according to our regimen reduced the treatment cost by Rs. 52120 for each patient- month of follow up.

Conclusion: IFX therapy followed by cDMARDs can prolong time to disease flare and decrease requirement for additional IFX dose in SpA. In the Indian setting, this regimen could be is a cost-effective option for patient with SpA.


  P9 Category: Spondyloarthropathy Top


An analysis of clinical, laboratory and management profile of Axialspondyloarthropathy patients managed at a tertiary carecentre

V Vasdev1 , ArunHegde1 , G D Choudhury1 , Abhishekkumar1 , Arjun MN1 , K Kishore1 ;1 Army hospital R & R, New Delhi

Background: Axial spondyloarthritis (Ax SpA) is one of the common rheumatic disorders and frequently cause disability and morbidity diseases that are frequent causes of disability. There is lack of large scale studies defining demography and other characteristics of AxSpA from Indian subcontinent. In this descriptive study, demographic, clinical, laboratory and management profile of patients with AxSpA is analyzed from the meticulous records maintained at an Armed forces tertiary care centre over last 08 years.

Objective: To study the clinical, laboratory and management profile of 1565 AxSpa patients from the registry maintained at an Armed forces tertiary care centre.

Patients and methods: A registry of AxSpa patients is being maintained at Army hospital R & R, New Delhi. A retrospective analysis of medical records of 1665 AxSpA patients who reported to this hospital since 2008 was carried out.

Results: A total of 1665 AxSpA patients (1573 males and 92 females) were included in the study. The mean age of the patients included in the study and onset of illness was 35.2 and 25.1 years, respectively. HLA - B27 positivity was detected in 74.4% of patients. The extraspinal joints most frequently involved were hip (7.6%), Knee (8.7%) and ankle (9.1%). Enthesitis was documented in 68.4 % and uveitis was recorded in 0.5 % subjects. The mean BASDAI, BASFI and BASMI score of patients was 3.4, 4.2 and 4.7, respectively. Sacroilitis on pelvic radiograph was seen in 79 % and the rest were positive on Magnetic resonance imaging. Biologics were given to 23.5 % of subjects with a mean BASDAI of 4.7. Anti TNF biologic agents used were Infliximab, Etanercept, Adalimumab and Golimumab in 304, 76, 06 and 04 subjects, respectively.



Conclusions: This study is first of its kind defining the clinical profile, laboratory and management characteristics of a large cohort of Indian AxSpA patients.


  P10 Category: Spondyloarthropathy Top


Outcome of Biologic therapy in patients with axial SpA - Experience from a tertiary care center

Vignesh Mantharam, Rajeswari Sankaralingam, Tamilselvam Tiruchengode Natesan, Sowndhariya Velu Annamalai, Sivakumar Vengudusamy, Bhuvanesh Mahendran, Ramesh R.; Institute Of Rheumatology, Madras Medical College, Chennai, India

Background
: Therapy of axial spondyloarthritis(SpA) has advanced with the advent of TNF inhibitors(TNFi). We assessed the outcome of TNF inhibitors in patients with axial SpA in a tertiary care center.

Methods: We conducted an open labelled prospective study between Feb 2015 and Feb 2016. Patients with axial SpA based on 2010 ASAS Classification criteria, of age 18-40 years, who did not respond to NSAID therapy were recruited. Patients who had contraindications to biologic therapy were excluded. Patients who met the inclusion and exclusion criteria were given either infliximab (3mg/kg iv, 0, 2 and 6 weeks) or 50 mg sc weekly etanercept (12 doses). Data including enthesitis, BASDAI, BASFI, BASMI, ASDAS-ESR and ASDAS-CRP were recorded at baseline, 12 weeks and 24 weeks. ASAS 20 and 40 were calculated to quantify definite clinical improvement.

Results: After obtaining clearance from Institute Ethics Committee, screening of 140 patients with axial SpA was done. 45 patients did not respond to NSAID therapy and 9 patients were excluded due to contraindications for TNFi. 25 patients received infliximab and 11 patients received etanercept. Baseline characteristics of were similar between the infliximab and etanercept arms (p=NS). As compared to baseline, both infliximab and etanercept therapies were associated with significant decline in enthesitis, BASDAI, BASFI, BASMI and good ASAS20 and ASAS40 at 12 weeks(p<0.05) as shown in table 1. However, no clinically significant difference of effect was observed between the two groups. After follow up period of 12 weeks without biologic therapy, reassessment done at 24 weeks showed significant increase in BASDAI in etanercept arm(2.78±1.17 to 4.25±0.95; p<0.05) than in infliximab arm (2.94±0.96 to 3.02±1.30; p=NS)

Conclusion: Low dose infliximab was associated with better clinical response at 12 weeks comparable with etanercept. It was associated with lesser increase in disease activity at 24 weeks than in etanercept arm.


  P11 Category: Spondyloarthropathy Top


Utility of CT in differentiating sacroiliitis associated with spondyloarthritis from gouty sacroiliitis

Jyoti Panwar1 , PulukoolSandhya2 , Aswin M Nair2 , Madhavi K1 , VisaliJeyaseelan3 , Debashish Danda2; Department of Radiology, Department of Rheumatology, Department of Biostatistics, Christian Medical College, Vellore

Background: Sacroiliitis though characteristic, is not unique to SpA. Sacroiliitis on imaging studies have prompted erroneous diagnosis of SpA in patients with gout. Our objective was to study the feasibility of distinguishing between gouty sacroiliitis and sacroiliitis associated with SpA using Computed Tomography (CT) imaging.

Methods: Cross-sectional retrospective study was undertaken for years 2010-2015. CT images of patients diagnosed to have sacroiliitis and clinical diagnosis of gout or SpAwere independently reviewed by two radiologists who were blinded for the diagnosis. Serial oblique axial and coronal images were analyzed for location, number, size and characteristics of erosion; tophi; subchondral sclerosis; joint space and ankylosis. The Receiver Operator Characteristic curve was constructed to analyze the discriminating ability of relevant features in identifying gouty sacroiliitis.

Results: CT-SI joint images of 11 patients with gouty arthritis and 224 patients with SpA were included. There was excellent correlation between the radiologists for all parameters. Erosions were numerous in SpA as compared to gout. 65.7 % (73/111) of erosions in gout were associated with tophi. As compared to SpA, higher number of erosions in gout were para-articular, had sclerotic margins, overhanging edges and multilobulated base (P < 0.0001 for all). Length and depth of erosions were more in gout as compared to SpA. Area under curve for length, depth of erosions and subchondral sclerosis was 0.665, 0.694 and 0.991, respectively. Subchondral sclerosis less than or equal to 4 mm had a sensitivity and specificity of 81.8% and 97.8%, for diagnosis of gout.

Conclusion: In addition to tophus, dimensions of erosions and characteristic features of erosions such as marginal sclerosis, multilobulated base, para-articular erosions and overhanging edges, absence of subchondral sclerosis is a unique feature that can distinguish sacroiliitis in SpA from gouty sacroiliitis.


  P12 Category: Spondyloarthropathy Top


Clinical features of Spondyloarthritis in females present differently. A myth or fact!

Sowndhariya V Annamalai, Rajeswari S, Tamilselvam T.N, Sivakumar V, Vignesh M, Mythili S ;Institute of Rheumatology, Madras Medical College, Chennai, India

Background: Spondyloarthritis, a group of interrelated inflammatory disorders with overlapping clinical manifestations along with shared genetic markers was once considered to occur predominantly in males. Now the gender ratio has become almost equal due to heightened awareness of its occurrence in females and better access to diagnostic tests.

Objectives:
To observe the differences in clinical picture of spondyloarthritis at first presentation in females in comparison with males including genetic marker HLA B27.

Results:
31 males and 31 females with features of spondyloarthritis at first presentation to our clinic are enrolled and their clinical characteristics were analysed. Mean age of presentation in females was 36.8±9.8 years whereas 30.9±8.6 years in males, this was statistically different (p=0.007). Median disease duration in females is 12 months whereas in males is 24 months. 58% of females had axial manifestations where as 87% of males had axial involvement (p=0.01). 83.8% of females had peripheral joint involvement whereas only 41.9% of males had peripheral involvement (p=0.001) at presentation. Only 19.3% of females were positive for HLA B27 in comparison with 54.8% of males (p=0.04). No significant difference was observed in occurrence of enthesitis, dactylitis, uveitis. No significant differences in BASDAI severity, ASDAS CRP, mean BASFI, mean BASMI and radiographic scoring of spine were observed in those with axial manifestations in the two groups. Psoriatic arthritis was the predominant type followed by undifferentiated spondyloarthritis in females whereas Primary Ankylosing spondylitis was the predominant subtype followed by Undifferentiated variety in males.

Conclusion:
This study reveals significant difference in age at presentation with females presenting at a later age than males. Axial manifestations predominated in males and peripheral manifestations predominated in females. HLA B27 was positive predominantly in males. Psoriatic arthritis was the predominant type in females whereas primary AS in males.


  P13 Category: Spondyloarthropathy Top


Psoriatic Arthritis - Clinical Profile And Outcome From A Tertiary Care Centre In India- 5 Years Experience

Sivakumar V, Rajeswari S, Tamilselvam T.N, Ramesh R, BalajiC,Vignesh M, Sowndhariya V.A, Mythili S, Kumudha M; Institute of Rheumatology, Madras Medical College, Chennai

Background: The clinical patterns of psoriatic arthritis varies in different parts of the world and there is little clinical and epidemiological data from India.

Aim:
To evaluate the clinical patterns and outcome of patients with psoriatic arthritis on DMARDs in a tertiary care centre.

Methods:
A retrospective observational study in which 124 patients of psoriatic arthritis fulfilling CASPAR criteria were included. Demographic patterns and disease parameters for minimal disease activity index were noted.

Results:
The patterns observed were symmetrical polyarthritis (69.35%) followed by asymmetric oligoarthritis (15.32%), predominant spondylitis (8.9%), predominant DIP arthritis (5.64%) and arthritis mutilans (1%). Enthesitis and dactylitis were present in 85.48% & 58.87% respectively. Nail changes were present in 82% of cases. Family history of psoriasis and psoriatic arthritis was present in 14.5% and 5.6% respectively. Minimal disease activity index(MDAI) was attained by 52.33%, 56.02%, 48.73% of patients at 6 months, 1 year and 5 years respectively(p<0.05).

Conclusion:
In contrast to the literature from the various other regions of the world where asymmetrical oligoarthritis is the most common pattern, symmetrical polyarthritis was the most common pattern observed in our study population concurring with various other studies from different regions of India. In spite of optimum therapy majority of the patients continued to have low disease activity.


  P14 Category: Spondyloarthropathy Top


Rheumatoid Arthritis and Seronegative Spondyloarthritis Overlap : Mirror, Myth or Mess

Kiran A, A Venugopalan, A Chopra; Centre for Rheumatic diseases, Pune,Maharashtra

Background : Historically and clinically rheumatoid arthritis (RA) and seronegativespondyloarthritis (SSA) are considered to be distinct end of spectrum disorders. However, it is often difficult to differentiate the two phenotypes. Despite limitation in interpretation, RF and HLA B27 continue to rule the diagnostic/classification requirements of these two disorders. What if they co-exist? Is there an overlap distinct phenotype? Does the overlap behave different in time compared to its individual components? However, no data exists to endorse this clinical claim. We have shown in the past that seropositivity for RF may be 95% and HLA B27 positivity (microlymphocytotoxicity assay) is found be 66%.

Aims : The current study is a retrospective audit of patients with overlapping clinical features of RA and SSA.

Methods: Records of 51,133 patients entered into the electronic database in a rheumatology referral center [Center for Rheumatic Diseases (CRD), Pune, INDIA] were screened. Standard rheumatology case record forms had been used to enter clinical features in database. Rheumatoid Factor(RF) and C-reactive protein (CRP) (Nephelometry), anti-cyclic citrullinated peptide (CCP) antibodies(2nd generation ELISA) and HLA-B27 (PCR-SSP) were performed apart from routine haematology, biochemistry and serology. 88 patients in the database had been given a provisional clinical diagnosis of a RA-SSA overlap at the initial examination by the rheumatologist.

Results : Among the 88 patients [Male:Female=1.3:1; Mean age at disease onset = 40 years; family history of inflammaroty arthritis = 37], 52 fulfill the ACR/EULAR 2010 criteria for RA (Arthritis Rheum 2010;62:2569) and/or ASAS criteria for SSA (Ann Rheum Dis, 2009,68;777). The phenotype of this cohort is described in the table below. None had occulo-mucocutaneous or any other extra-musculoskeletal feature.



Out of the remaining 36 patients 31 patients continued to have a phenotype of RA-SSA overlap and in 5 it seemed to be an error of clinical judgement. All the 31 with clinical overlap satisfied the ACR/EULAR 2010 criteria for RA but in addition all showed some typical clinical characteristics of SSA (21 inflammatory spondylitis with/without sacroilitis, 8 only sacroilitis and 2 only with enthesitis/dactylitis.

Conclusion
: We describe the uncommonly occurring phenotype of RA-SSA overlap satisfying standard criteria of both the constituent disorders. The overlap needs to be investigated further for distinct immunogenetic markers. We also need to explore the treatment response and prognosis of the overlap syndrome.


  P15 Category: Spondyloarthropathy Top


HLA-B27 POSITIVITY IN SERONEGATIVE SPONDYLOARTHRITIS: A HOSPITAL BASED CROSS-SECTIONAL STUDY

D Timung, P Dihingia, S Das, S M Baruah, T K DAS, C Dutta; Department of Medicine, Assam Medical College, Dibrugarh

Background: The seronegativespondyloarthritis are a group of disorders involving the axial skeleton and having seronegative status and sharing certain clinical features and genetic associations as well as pathogenic mechanisms. It includes Ankylosing Spondylitis, Reactive Arthritis, Psoriatic Arthritis and Spondylitis, Enteropathic Arthritis and Spondylitis, Juvenile onset Spondyloarthritis and undifferentiated Spondyloarthritis. They are associated to varying extents with HLA-B2. The study was conducted to examine HLA-B27 positivity in Spondyloarthritisin upper Assam of North-East India

Materials and Methods: The present study is a hospital based cross-sectional study, carried out on 129 patients of SeronegativeSpondyloarthropathy, diagnosed according to ASAS,2009 criteria, over a period of one year from July, 2014 to June, 2015. Seropositive Arthritis, Rheumatoid arthritis, cancer, pregnancy, sepsis, recent trauma and crystal arthropathy were excluded from the study. A detailed evaluation of clinical history, physical examination and investigations and the results were recorded in a predesigned proforma. The study was approved by the Institutional Ethical Committee.

Results: Out of the 129 patients, 97(75.19%) were male and 32(24.81%) were female. Ankylosing Spondyloarthritis (53.49%) is the most common in both male and female (p <0.05). Median age of presentation was 28 years. The Inflammatory back pain was the most common clinical manifestation in the different types of SpA(p<0.05). HLA-B27 was positive in 79(61.24%) and highest number of positivity was seen in Ankylosing spondylitis. Highest score of BASDAI, BASFI, BASMI, HADS and sacroilitis were most in Ankylosing Spondylitis and HLA-27 positive cases.

Conclusion : Ankylosing Spondylitis is the most common Spondyloarthritis with highest positive likelihood ratio in terms of pretest and post test probabilities of HLA-B27. All the disease severity criteria were higher in HLA-B27 positive cases. High BASDAI, BASFI, HADS score with high disease activity had greater work limitation.


  P16 Category: Spondyloarthropathy Top


Gender differences in patients of Spondyloarthritides

Dr. Nibha Jain1 , Dr. Mitesh Makwana1, Dr. Sapan Pandya2 Dr. Puja Srivastava2 ; V.S. General Hospital, Ahmedabad

Background/Purpose: To study the demographic and clinical profile of patients of SpA and to look at gender differences, if any.

Methods : Study period was from Nov 1st 2015 to august 30th 2016. All consecutive patients with SpA were included in the analysis. Descriptive statistics was applied

Results: Mean age was 30.8 + 11.1. The profile matched those from other centers from India and elsewhere. While dactylitis was commoner in females, more males had ankylosing spondylitis with high CRPs and HLAB27 positivity.

Conclusion: The profile of patients presenting with SpA matched with those from other centers. More numbers would be needed to look at gender differences in this group of diseases




  P17 Category: Spondyloarthropathy Top


Clinical Profile of patients with peripheral spondyloarthritis in a tertiary care hospital

Hegde Arun, Vasdev Vivek, Abhishek Kumar, Arjun MN, Kishore Kunal, Choudhury GD; Army Hospital Research & Referral, New Delhi

Purpose:To study the clinical profile of peripheral spondyloarthritis in 100 adult males presenting to a tertiary care hospital.

Methodology:
Average age of patients was 30 years. Average disease duration prior to presenting to the hospital was 6 months. The knee joint was involved in 93% patients, ankle in 49% patients, wrist in 17% patients and Hip jt in 1% of patients. 12% of patients had dactylitis and 17% of patients had enthesitis (12 patients had heel enthesits, 3 patients had enthesitis at tendo Achilles while 2 patients had chest enthesitis).6% patients had skin involvement (5 patients had balanitis, 3 had keratoderma blenorrhagicum and 1 had erythema nodosum). Average ESR was 28 mm fall by Westergen method. 27% patients were HLA B27 positive. 56% patients required DMARDs in form of sulfasalazine.3 patients required methotrexate in addition to sulfasalazine for control of peripheral arthritis.11% patients had bilateral sacroilitis on imaging at initial presentation.4% patients went on to require biological agents, out of which 3 patients received Etanercept and 1 patient received Infliximab.

Conclusion:
Peripheral spondyloarthritis is a distinct entity in itself among the spondyloarthritis requiring DMARDs in the majority of patients. However in our experience the requirement of biologics for disease control was miniscule.


  P18 Category: Spondyloarthropathy Top


A single center retrospective analysis of axial spondyloarthritis patients

Anuj Singhal1 , Sudhir K Joshi2 , V Hande3 , R Tyagi4; Department of Rheumatology, Dept of Cardiology, Dept of Medicinie,Pulmonologist, INHS Asvini, Mumbai, Maharashtra, India

Background : Axial spondyloarthritis (AxSpA) is a chronic inflammatory disease routinely treated with nonsteroidal anti-inflammatory drugs (NSAIDs). Patients unresponsive to NSAIDs are treated with tumor necrosis factor (TNF-α) inhibitors.

Purpose: To evaluate the change in AxSpA disease activity in response to various treatments.

Methods : Medical records of AxSpA patients (n=130), who visited our center between January 2014 and June 2016, were analyzed, retrospectively. All patients were initially treated with NSAIDs while those who had uveitis also received sulfasalazine (n=40) therapy. Patients unresponsive to NSAIDs were shifted to anti-TNF-α biologics-etanercept (n=13) or infliximab (n=17). The changes in disease activity in response to treatment were assessed by recording Bath Ankylosing Spondylitis Disease Activity (BASDAI) score and Ankylosing Spondylitis Disease Activity Score (ASDAS) Erythrocyte Sedimentation Rate (ESR), at 0, 6, and 12 months.

Results : Majority of the patients were males (N=121, 93%) and the mean age of the analysis population was 31.5±8.2 years. Clinical presentation and diagnostic parameters are presented in Table 1. Both BASDAI score and ASDAS ESR steadily decreased from baseline to 6 and 12 months after treatment (ANOVA, p<0.0001; Figure 1).

Conclusion : Results of this study showed that the disease activity in AxSpA patients reduced from baseline to 12 months in response to the different treatment approaches.


  P19 Category: Spondyloarthropathy Top


Use of Golimumab in Indian patients with Ankylosing Spondylitis: A single centre experience

Arun Gogna, Sameer Gulati; Vardhman Mahavir Medical College and associated Safdarjung Hospital, New Delhi - 110 029



Background: Tumor necrosis factor (TNF) blocking agents are recommended to patients with persistently high disease activity despite treatment with NSAIDs. Small single centre studies from India have found statistically significant reduction in mean BASDAI and ASDAS-ESR scores in AS patients with use of etanercept and infliximab. However, similar experience with use of Golimumab on Indian AS patients is lacking.

Aim: To observe the clinical efficacy of Golimumab at 12 weeks of therapy on Indian AS patients with NSAID therapeutic failure.

Materials and Methods: We retrospectively analyzed AS patients visiting our Rheumatology services who were being treated with 50 mg Golimumab subcutaneously once a month. The AS patients who failed to show improvement with adequate therapeutic trial of NSAIDs or had a loss of response to Infliximab were offered Golimumab. Active infections, active/latent tuberculosis, congestive heart failure, pregnancy, breast feeding and malignancies were ruled out before starting Golimumab. Patient reported outcomes concerning back pain, peripheral pain/swelling, duration of morning stiffness and patient global assessment of disease activity were recorded at baseline and after 12 weeks of therapy. C Reactive Protein (CRP) and HLA B27 were obtained along with relevant radio imaging. Baseline and 12 weeks post therapy ASDAS-CRP were calculated as outcome measure for assessment of therapeutic response. A change in the ASDAS-CRP score of 1.1 and 2.0 were considered as "clinically important" and "major improvement" respectively.

Results : Four patients (3 male and 1 female), with a mean age of 46.7 ± 14.5 years, were started on Golimumab. The mean duration of symptoms were for 12.1 ± 7.2 years (range: 2.5 to 20 years). Mean ASDAS-CRP was 4.43 ± 0.91 (range: 3.66 - 5.66). Axial involvement was seen in 3 patients. The remaining 1 patient had both axial and peripheral joints involvement. Significantly, all the four patients had radiographic evidence of sacroiliitis. Only one patient was HLA B27 negative, the remaining 3 were positive. One patient was started on Golimumab after failing Infliximab therapy. "Major improvement" in ASDAS-CRP score was seen in all the 4 patients.

Conclusions : Golimumab leads to major improvement in Indian AS patients at 12 weeks post therapy. It may also be offered to AS patients with Infliximab failure. Our study may be taken as a pilot study to plan further research with larger sample size.


  P20 Category: Spondyloarthropathy Top


Infliximab in Ankylosing Spondylitis

Kakade Girish, Samant Rohini, Sagdeo Parikshit, Shah Romi, Joshi Manohar; P D Hinduja National hospital & Medical Research Centre, Mumbai.

Introduction: TNF- alpha inhibitors are used in patients with ankylosing spondylitis, having inadequate response to NSAIDs and/or conventional DMARDs, especially in axial disease. Infliximab is the most commonly used TNF blocker.

Objective: To describe clinical profile and outcome of infliximab in patients with ankylosing spondylitis.

Methods: This was a retrospective study in which data of patients of AS who received infliximab was recorded from medical record files. Demographic profile, clinical features, use of other DMARDs, dosage & interval between infliximab doses, primary/secondary failure & adverse events were recorded.

Results: Data of 10 patients (7 males & 3 females) were recorded. Mean age at diagnosis was 29.4 yrs. Mean duration of AS was 10.45 yrs. Axial involvement was seen in all 10 patients. Peripheral joint involvement was seen in 5/10 patients. Mean interval between diagnosis of AS to infliximab was 58.2 months. Mean follow up after first dose of infliximab was 33.5 months. 8 patients were receiving concomitant conventional DMARDs along with infliximab (3 methotrexate & 5 sulphasalazine). 1 patient had secondary failure to etanercept earlier. Mean dose & mean interval between doses were 3.47 mg/kg & 5.10 months respectively. Primary & secondary failures were seen in 0 & 4 patients respectively. Of 4 patients with secondary failure,3 were females & 3 were receiving either methotrexate/ sulphasalazine concomitantly. Mean dose & mean interval between doses were 4.07 mg/kg & 5.48 months respectively in secondary failure group. Mean number of doses prior to secondary failure were 6.75. There were 2 serious adverse events recorded. 1 patient had serious infusion reaction, while another patient developed pulmonary koch's.

Conclusion: All patients had adequate primary response to infliximab. Majority patients with secondary failure were females (3/4). Inadequate dosages & larger intervals between 2 doses of infliximab may be contributing to secondary failure.


  P21 Category: Spondyloarthropathy Top


A Study of Clinical Profile in Male and Female Spondyloarthropathy

Laxmi Patil, Jyotsna Oak; Department of General Medicine and Rheumatology, Kokilaben Dhirubhai Ambani Hospital, India

Background/Purpose: Ankylosing Spondylitis is more common in males and is not considered as differential diagnosis in females with inflammatory low backache.This leads to delay in diagnosis as well as treatment. There are very few studies done regarding female spondyloarthropathy. There are few studies on female SpA patients in Indian population to understand the clinical profile, mode of presentation and course of their disease.

Methods:
60 males and 60 females satisfying ESSG or ASAS criteria for diagnosis of spondyoarthropathy were included in the study. Demographic characteristics like age, sex, age at time of onset of symptoms, age at diagnosis, diagnostic delay were recorded. Clinical data included articular and extrarticular symptoms, family history was collected. Clinical Assessment Scoring was done for all patients using BASDAI, BASFI, BAS- G, BASMI indices. Investigations like ESR, HLA B27, X-ray and/or MRI SI joint reports were noted. Data was analysed using unpaired T test for continuous data and chi square test for categorical data. P<0.05 was considered as significant.

Results: Age of onset of symptoms (M: 25.7±7.3yrs; F:28.3±9.9yrs) and age at first presentation (M: 30.20±9.21yrs ; F: 31.28±10.8yrs) in females was greater than in males. Clinical Assessment with BATH indices showed females had equally severe disease as in men. (Table.1). Most common presenting symptom being low back pain followed by peripheral arthritis. It was observed that Indians have peripheral arthritis more common than the western population. However there was no significant difference in the delay in diagnosis in both the groups.

Conclusion: The severity of symptoms and disability is same in females and males with SpA. Females were increasingly diagnosed with SpA. Peripheral arthritis predominantly lower limb oligoarthritis is common in Indian population compared with western population. Most common symptom of presentation was low back pain and second most common symptom a gluteal pain.




  P22 Category: Spondyloarthropathy Top


Acute Myeloid Leukaemia masquerading as a case of peripheral spondyloarthritis in an adult male

Hegde Arun, Kumar Abhishek, Vasdev Vivek, MN Arjun, Kishore Kunal, Choudhury GD; Army Hospital Research & Referral, New Delhi

Background: Arthritis is a known presenting feature in haematological malignancies of childhood. However haematological malignancies presenting as arthritis in adults are rare. Herein we report the case of a 42 year old man with acute myeloid leukaemia (AML) who presented with features of peripheral spondyloarthritis as initial manifestation.

Case Report: This 42 years old patient presented with fever and lower limb oligoarthritis associated with leucopenia. He was treated initially as reactive arthritis, however due to progression of arthritis, rheumatology opinion was sought. Clinical examination revealed enlarged left supraclavicular lymph nodes, Rt knee synovitis and bilateral tendoachilles enthesitis. Initial investigations revealed bicytopenia, with normal peripheral blood smear and a reactive bone marrow on aspirate.CT chest and abdomen were unremarkable. Excision biopsy of left supraclavicular lymph node revealed reactive lymph node with paracortical hyperplasia and features of macrophage activation. Evaluation for arthritis revealed raised acute phase reactants and positive HLA B27 status. MRI of the sacroiliac joints was normal. The patient fulfilled clinical criteria of peripheral spondyloarthritis and was started on sulfasalazine. Patient showed partial improvement in clinical and haematological parameters, however, two weeks later patient had recurrence of fever with extreme prostration. Clinical examination did not reveal any fresh finding. Investigations revealed anemia, leucocytosis and immature leucocytes on peripheral blood smear. Repeat bone marrow aspiration and karyotyping revealed features of acute myeloid leukaemia(M0 subtype). The Pt was started on induction therapy which was followed by resolution of arthritis.

Conclusion:
Leukaemic arthritis defined as joint pain and swelling in association with peripheral blood or bone marrow leukaemia, is secondary to infiltration of joints by leukemic cells and is more common with ALL than AML. Childhood leukaemias commonly present with arthritis, however, a high degree of suspicion is required in adult patients presenting with arthritis and cytopenias.


  P23 Category: Rheumatoid Arthritis Top


Effect of seasons, diet & lunar cycles on disease flare in Rheumatoid Arthritis(RA) - A patients perspective

Sofia Mohan; Lingala Joel Prathap Raju; Dr. John Mathew; Christian Medical College, Vellore

Background/Purpose: Patients complain of flare of symptoms with seasonal changes in weather, variation in dietary factors & changes in the lunar cycle. So we studied the significance of the influence of these factors on patients with RA.

Methods: A prospective cross-sectional study was done from March to November 2015. Patients with RA fulfilling the ACR/EULAR 2010 criteria and consenting were given the questionnaire and interviewed. A visual analog scale (VS) was used to document the difference in pain, if any. The sample size was 25, for a power of 80% and an error of 5%. SPSS 16 was used, with independent t-test for assessing a statistically significant difference in pain.

Results: 115 patients were seen. Of the 114 from whom data was collected, 12 patients did not fit into the inclusion criteria and were excluded.

  1. Of the 68(66.7%) who stated winter as an exacerbating factor, the increase in pain scale between summer and winter was statistically significant. (p value - <.001)
  2. 38 (37.3%) patients who experienced adverse effects with certain foodstuff had significant pain relief on stopping consumption of the said food (p value - <.001). The exacerbation in pain was found to be significant. 28 named vegetarian food and 10 named non-vegetarian food.
  3. Only 13 (12.7%) experienced exacerbations at either extremes of the lunar cycle i.e new moon or full moon. The number of patients with exacerbations with phases of the lunar cycle was not statistically significant. (p value -.052). The subjects who experienced variation with lunar cycle had significant increase in pain. (p value - <.001)




Conclusion:- Ours is the first study done in tropical/developing countries to document seasonal flare and vegetarian diet as a predominant cause of flare in RA.


  P24 Category: Rheumatoid Arthritis Top


Assessment of Methotrexate induced hepatotoxicity by transient elastography (Fibroscan®) in Rheumatoid arthritis patients : a cross sectional study.

V Vasdev, Abhishek Kumar, Arun Hegde, G D Choudhury, Arjun MN, K Kishore; Army hospital R & R, New Delhi

Background: Methotrexate (MTX) is the pillar of pharmacotherapy of Rheumatoid Arthritis (RA) which is the most common inflammatory arthritis. MTX is known to cause liver fibrosis and periodic liver function tests are recommended with long term use. The gold standard test for detecting liver fibrosis has been liver biopsy, which is a procedure not bereft of potential serious complications. Use of transient elastography (Fibroscan) has turned out be a reliable non-invasive method of assessing liver fibrosis.

Objective: To assess hepatotoxicity and liver fibrosis in RA patients on long term MTX therapy.

Patients and Methods: It was a cross-sectional study including patients with RA on long term MTX therapy (>5 yrs). The cumulative MTX dose was calculated from case records. Serial transaminase levels for the entire duration of MTX therapy were obtained from case records. Hepatic stiffness was determined by FibroScan and other liver function tests (LFT) including serum bilirubin, serum transaminases, total proteins, albumin and prothrombin time were also carried out.. All patients were evaluated for comorbid conditions known to cause liver fibrosis and were excluded from the study.

Results: A total of 160 patients(134 females and 26 males) were included in the study. Mean age of the patients was 54.64 years (range 23 - 77 years). Mean duration of methotrexate use was 6.5 years. Mean MTX cumulative dose was 4942 mg (range 2040-18200 mg). Mean hepatic stiffness was 4.8 (Range 9.0 - 2.0)KPa. No patient had hepatic stiffness qualifying for a liver biopsy. None of the patient had significant derangement in other LFTs..

Conclusions: Long term MTX use in RA is safe with none of the patients showed significant liver fibrosis or liver function derangements.


  P25 Category: Rheumatoid Arthritis Top


Serum CXCL16 levels in seropositive rheumatoid arthritis patients before and after treatment with DMARDS

Ramesh Ramamoorthy, Rajeswari Shankaralingam, Balaji Chilukuri, Tiruchengode Natesan ; Tamilselvam, Selvakumar Balameena, Sowndhariya Velu Annamalai, Vignesh Mantharam; Institute of Rheumatology, Madras Medical College, Chennai, India

Background:
Rheumatoid arthritis (RA) is characterized by profound mononuclear cell (MNC) recruitment into synovial tissue (ST) with CXCL16 being the prime MNC recruiter. Hence, changes in serum CXCL16 levels can be used as a prognostic marker in RA.

Aim
: To study the levels of serum CXCL16 in seropositive RA patients(either RF+ or ACPA+ ) before and after treatment with DMARD (both conventional synthetic and biologic)

Materials and Methods:
After obtaining Institutional ethical clearance and written informed consent from patients, a prospective observational study was conducted among 31 RA patients. All patients were initially treated with Hydroxychloroquine, methotrexate and sulfasalazine for 3months, failing which, they were shifted to anti TNF biologics (n=5). Serum CXCL16 levels were measured using ELISA at baseline and after 6months. 18 age and sex matched controls was taken.

Results:
26 patients showed a lowering of mean serum CXCL16 levels from 56.07pg/ml to 21.79pg/ml (62% reduction) after 6 months of conventional synthetic DMARDs. Of the 5 patients who were treated with TNF-α blocker had their CXCL16 levels reduced from 63.81pg/ml to 12.36pg/ml (80.6% reduction) at 6 months. There was a corresponding improvement in the disease activity of RA. Mean DAS 28 before treatment is 5.2 and after treatment is 2.1. (p=0.001). Lowering of CXCL16 was found to correlate positively with clinical symptoms and lowering of disease activity. r=0.97; p=0,001.

Conclusion:
DMARDs treatment significantly lowered the serum levels of CXCL16 in patients with RA (more with biologics than csDMARDs) which correlates with clinical improvement. Hence, CXCL16 can be used as a marker for response to treatment in RA patients.


  P26 Category: Rheumatoid Arthritis Top


Clinical efficacy and safety of interleukin-6 receptor antagonist (Tocilizumab) therapy in Indian seropositive rheumatoid arthritis patients

Abhishek Kumar, Vivek Vasdev, GD Choudhury, Arun Hegde, Arjun MN; Department of Rheumatology, Army Hospital (Research & Referral) Delhi cantt

Background: Interleukin-6 receptor antagonist (Tocilizumab) is being used in India for RA at multiple centers, however, there has been no study which has reported its safety and efficacy in Indian patients.

Method:
This prospective observational study was conducted at a tertiary care rheumatology centre over a period of 24 weeks. All adult seropositive RA patients (>16 years age) who continued to have persistent moderate to high disease activity (DAS28-ESR score≥ 3.2) despite treatment with at least two non-biological DMARDs (one of which should have been methotrexate) taken for at least 3 months, were included in the study, after taking informed consent. All patients received Inj Tocilizumab (8mg/kg body weight) at baseline and at 04 weeks interval up to a period of 6 months. The patients were followed up at 4 weeks interval till 24 weeks and at every visit various clinical and laboratory parameters were assessed as per protocol. The Primary outcome was EULAR response at 26 weeks and secondary outcomes were changes in acute phase reactants and drug related adverse reactions.

Results: A total 42 patients were included in the study. The mean age of patients was 46.63 years. Mean duration of disease of 7.2 years. EULAR good and moderate response was achieved by 29 (69.04%) and 6 (14%) patients respectively at the end of the study. The mean DAS28-ESR and DAS28-CRP reduced significantly from the baseline value (DAS28- ESR 5.2 to 2.2, 95% CI 2.30 to 3.01), p value <0.005; DAS28-CRP 4.9 to 2.1, 95% CI 2.2 to 2.72), p value <0.005). There was similar improvement in disability index at 24 weeks (HAQ-DI 1.7 to 0.33, 95% CI 1.16 to 0.59), p value <0.001). The adverse effects reported by patients were minor (Infections - 4 [9.52%], raised ALT level 5 [11.4%] and raised LDL level 7 [23.3%]).

Conclusion:
Tocilizumab is an effective and safe option for management of DAMRD refractory seropositive RA patients.


  P27 Category: Rheumatoid Arthritis Top


Exploring the potential effect of HMG-CoA reductase inhibiton and Angiotensin receptor Blockade on Vascular Function in Rheumatoid Arthritis

Nidhi Garga , Ashit Syngleb , Pawan Krishanc ; Chitkara College of Pharmacy, Chitkara University, Punjab, India. Cardio Rheuma & Healing Touch City Clinic, Chandigarh & Consultant Rheumatologist- Fortis Multi Speciality Hospital, Mohali; Department of Pharmaceutical Sciences & Drug Research, Punjabi University, Patiala, Punjab, India.

Background/Purpose: Increased cardiovascular risk in rheumatoid arthritis (RA) provides a strong rationale for early therapeutical interventions. In view of the proven benefit of Statins and ARBs in atherosclerotic vascular disease, we aimed to delineate the impact of rosuvastatin and olmesartan on vascular function in RA.

Methods: 82 RA patients randomized into 3 groups to receive 24 weeks of treatment with rosuvastatin (10 mg/day, n=28), olmesartan (10 mg/day, n=26) and placebo (n=28) as an adjunct to existing stable antirheumatic drugs. FMD assessed by AngioDefender. EPCs estimated by flow cytometry. DAS28, CRP and ESR, pro-inflammatory cytokines (TNF-α, IL-6 and IL-1), serum nitrite, TBARS, ICAM-1, VCAM-1 and lipids measured at baseline and after treatment. The SCORE system estimates the 10 year risk of first fatal atherosclerotic event. Quality of life assessed with HAQ-DI and SF-36.

Results: After 24 weeks, FMD and EPCs levels were increased and nitrite and TBARS levels were decreased significantly in both rosuvastatin and olmesartan groups, respectively, compared with placebo. Both rosuvastatin and olmesartan significantly decreased DAS28, ESR, CRP and TNF-α as compared with placebo. Rosuvastatin also significantly improved IL-6, ICAM-1 concentration but olmesartan significantly improved IL-1, VCAM-1 concentration. There is significant reduction in the SCORE and quality of life after both treatment groups as compared with placebo. After treatment with rosuvastatin, FMD correlated inversely with TNF-α and CRP, in olmesartan group FMD inversely correlated with CRP.

Conclusion: First study to show that rosuvastatin and olmesartan improve vascular function in RA. Both the rosuvastatin and olmesartan increases the EPCs, lowers TNF-α which down regulates the production of CRP, NO and TBARS which improves the inflammation and endothelial dysfunction. However, Rosuvastatin also favourably impacted ICAM-1 and olmesartan has beneficial effect on VCAM-1. Thus, both rosuvastatin and olmesartan have anti-inflammatory and vasculoprotective effects in RA mediated through anti-proinflammatory cytokine action


  P28 Category: Rheumatoid Arthritis Top


Genetic And Expression Changes In Tnf-Α As A Risk Factor In Rheumatoid Arthritis Pathogenesis In Northeast India

Somdatta Das1 , Chitralekha Baruah2 , Anjan Kumar Saikia3 , Sujoy Bose1, 4 ;1 Department of Bio-engineering and Technology, Gauhati University, Guwahati.2 Department of Medicine, Gauhati Medical College & Hospital, Guwahati3 Department of Gastroenterology, Hayat Hospital, Guwahati4 Department of Biotechnology,Gauhati University, Guwahati

Background: Although anti-tumor necrosis factor-alpha (TNF-α) therapy is used as a clinical intervention for rheumatoid arthritis (RA), but differences exist in the response to the treatment; which makes the candidature of the screening of TNF-α alteration(s) at genetic and expression level an important agenda prior to treatment. Differences exists w.r.t available data in this regard globally, with no data from the ethnically distinct northeast Indian population.

Aim: To determine the associative role of TNF-α -308A/G polymorphism, and differential expression of TNF-α in the pathogenesis of RA.

Methods: A total of 60 RA patients were enrolled for the present study based on ACR and EULAR (2010) criteria, along with 40 community based age and sex matched healthy controls. TNF-α -308A/G polymorphism was screened by T-ARMS PCR assay. The differential expression level of TNF-α mRNA and protein level was quantified by using qPCR and ELISA respectively. Statistical analysis was performed using SPSS software.

Results: TNF-α -308 variant GA genotype was higher in RA cases (45%) than in controls (25%). Presence of TNF-α -308 variant A allele was associated with increased risk of RA susceptibility [OR=2.531 at 95%CI,p=0.056] and severity [OR=3.5 at 95%CI, p=0.160] in NE Indian patients. The mRNA expression level of TNF-α was upregulated in RA cases (avg 55.85±39.52folds) compared to controls. The TNF-α protein level was found to be higher in both mild (20.7±2.19pg/ml) and severe RA cases (23.2±1.1pg/ml) compared to controls (17.13±3.22pg/ml). Presence of TNF-α -308 variant GA genotype in RA cases was associated with higher TNF-α mRNA and protein expression.

Conclusion: Presence of TNF-α -308A allele is associated with increased risk of RA susceptibility, severity and differential TNF-α expression, and thus has prognostic signifi-cance. Higher TNF-α pro-inflammatory cytokine levels are associated with RA in NE Indian patients; making the population suitable subjects for anti-TNF-α therapy and its benefits.


  P29 Category: Rheumatoid Arthritis Top


Stem Cell Augmentation for Cardiovascular Risk in Rheumatoid Arthritis with Olmesartan: Star-O Study

Ashit Syngle1 , Nidhi Garg2 , Pawan Krishan3 ; Cardio Rheuma & Healing Touch City Clinic, Chandigarh & Consultant Rheumatologist- Fortis Multi Speciality Hospital, Mohali; Department of Pharmaceutical Sciences & Drug Research, Punjabi University, Patiala, Punjab

Background: Cardiovascular (CV) disease remains the leading cause of mortality in rheumatoid arthritis (RA). Endothelial progenitor cells (EPCs) are depleted and contribute to increased CV risk in RA. Olmesartan exerts a protective CV effect in diabetes by augmenting EPCs. However, this vasculoprotective effect of olmesartan has not yet been investigated in RA. We aimed to investigate the impact of olmesartan on EPCs in RA.

Methods : Forty RA patients fulfilling the 2010 Rheumatoid Arthritis classification criteria were randomized to receive 24 weeks of treatment with olmesartan (10mg/day, n=20) or placebo (n=20) as an adjunct to existing stable antirheumatic drugs. EPCs (CD34+/CD133+) quantified by Flow cytometry. Flow mediated dilatation (FMD) assessed by AngioDefender. Inflammatory measures included DAS28, CRP, ESR, pro-inflammatory cytokines (TNF-α, IL-6 and IL-1), serum nitrite and adhesion molecules (ICAM-1 and VCAM-1) measured at baseline and after 24 weeks.

Results: At baseline, inflammatory measures, pro-inflammatory cytokines, adhesion molecules and nitrite levels were elevated and EPCs and endothelial function were impaired among both groups. EPCs significantly 0.018±0.001 to 0.031±0.001 increased p<0.01 after treatment with olmesartan but did not show significant change with placebo 0.019±0.001 to 0.022±0.001, p=0.14. FMD improved significantly with olmesartan as compared to placebo. Olmesartan significantly decreased systolic/diastolic blood pressure (mean difference, -7.5/-1.6, p=0.01) as compared with placebo (mean difference, -4.1/-1.2, p>0.05). DAS28, ESR, CRP (all p=0.01) and nitrite (p=0.03) were significantly reduced after treatment with olmesartan. Proinflammatory cytokines TNF-α, IL-6 and VCAM-1 significantly (p=0.38, p=0.02 and 0.01 respectively) reduced in olmesartan group. Significant negative correlation was observed between EPCs and CRP, TNF-α, IL-6, VCAM-1 and FMD after treatment with olmesartan.

Conclusions: First study to show that olmesartan improves inflammation, EPC biology and endothelial dysfunction in RA possibly through its anti-inflammatory effect via inhibition of proinflammatory cytokines. This anti-inflammatory effect may be of therapeutic relevance in RA.


  P30 Category: Rheumatoid Arthritis Top


Effect of Periodontal infections on proinflammatory cytokines and PAD4 levels in patients with Rheumatoid Arthritis.

Sudipta Chatterjee, Sanchaita Misra, Pradyot Sinhamahapatra, Alakendu Ghosh; Institute of Post Graduate Medical Education and Research, Kolkata, India

Purpose : The contribution of periodontal infections in disease activity of Rheumatoid Arthritis (RA) has long been implicated. This study is directed towards understanding the crosstalk between RA and PD.

Methods: 30 consecutive RA patients, 15 with PD and 15 without PD were selected from those attending the Rheumatology OPD. Study also included 15 age and sex matched healthy controls. Patients were selected prior to antimicrobial as well as steroid therapy (2 months) and ones with other systemic involvements were excluded. Diagnosis for RA and PD were done following the ACR 1987 criteria (for RA) and British Society of Periodontology 2011 criteria (for PD). Serum samples were collected and were analysed for RF, anti-CCP, TNFα, IL1β, IL-17 and PAD4 by ELISA. CRP levels were measured by Nephelometry.

Results: TNFα levels significantly correlated (r =.04) with the DAS28 scores in all RA pateints. Higher disease activity was seen in RA with PD compared to RA without PD (DAS28: 4.535±1.058vs2.973±0.5395, p<0.03). TNFα level was significantly higher in RA patients with PD than those without PD (4210±1351vs720±315.4, p<0.0001) (Fig:A). Higher levels of PAD4 was also observed in RA patients with PD compared to RA without PD (225.3±37.17vs154.9±44.17, p<.003) (Fig:B).

Conclusion: Higher serum level of TNFα and higher DAS28 in RA with PD patients compared to RA without PD indicated the contribution of periodotitis in triggering systemic inflammation. Higher levels of PAD4 in RA with PD might be due to upregulation of the particular enzyme in periodontitis. Hence, mechanism of PAD4 upregulation in periodontitis is a subject of key research.




  P31 Category: Rheumatoid Arthritis Top


Waist circumference is an independent risk factor for metabolic syndrome in RA patients: A prospective study

Abdul Gafoor PK1 , PV Bhargavan1, 2 , Vinod Ravindran3, 4 ;1 Department of Medicine &3 Rheumatology, MES Medical College, Perinthalmanna,2 Department of Medicine, Baby Memorial Hospital, Calicut,4 Centre for Rheumatology, Calicut

Background: Patients with rheumatoid arthritis (RA) have higher prevalence of metabolic syndrome. In this study we appraised various risk factors for it among patients with RA.

Methods:
All consecutive adult patients were prospectively enrolled at diagnosis of RA. Metabolic syndrome was defined as per the National Cholesterol Education Program (NCEP) guidelines. Demographics, RA related characteristics and traditional cardio-vascular risk factors were recorded. Uni-variate and multi-variate logistic regression analyses were used to predict independent risk factors of metabolic syndrome.

Results :
100 patients (age, 48±11.3 years; 89% female; disease duration, 5±5.6 years; RF + 58%; DMARD naive 52%) were studied. Five patients were smokers (all males), 30% had hypertension and 17% were diabetic. Thirty- two patients had metabolic syndrome. In these patients a uni-variate analysis revealed RA characteristics of disease duration, ESR, and DAS28 were not significant predictors. In the multi-variate logistic regression analysis (Table 1) waist circumference (WC) was found to be the strongest independent risk factor.





Conclusion:
To reduce cardiovascular morbidity in patients with RA attention to due life style modification appears important based on the results of our study.


  P32 Category: Rheumatoid Arthritis Top


No impact of sero-negativity on the efficacy of biosimilar rituximab in biologic naïve patients with active rheumatoid arthritis

Biju Mohan1 , Vinod Ravindran1, 2 ;1 Department of Rheumatology, National Hospital, Calicut,2 Centre for Rheumatology, Calicut

Background : Rituximab (RTX) is widely used in the treatment of rheumatoid arthritis (RA). Previously we had shown that more affordable bio-similar version of RTX is efficacious in sero-positive RA.1 However the strategy to use RTX only in sero-positive patients excludes several who would potentially benefit from its long term efficacy. In this prospective study we appraised the usefulness of bio-similar RTX -Mabtas™ RA in sero-negative RA patients.

Methods : All willing consecutive adult patients with RA who continued to have active RA despite standard combination DMARDs therapy and were infused 2 doses of 1000mg of Mabtas™ RA were prospectively screened. Only those patients who were sero-negative for both rheumatoid factor and anti-citrullinated peptide antibodies (ACPA) were included in this analysis.

Results : Seventeen patients (mean age 55 ± 4.4 years; 67% females) were included with a follow up range of 6- 12 months. Two had interstitial lung disease (ILD). At 24 weeks compared to the baseline both DAS28 ESR (5.44± 0.18 vs 2.58± 0.76, p<0.0001) and HAQ (1.83 ±0.56 vs 1.12±0.29, p<0.0001) showed significant improvement. Sustained remission (mean range, 8 months) was noted in 15 and 2 had moderate but sustained response. De-escalation of existing therapy including of glucocorticoids was possible in 14 patients. No retreatment with Mabtas™ RA was required in the study period. No worsening of ILD was documented in those 2 patients. Infusion reactions were none and no SAEs were observed.

Conclusion: Sero-negativity did not have any impact on the efficacy of Mabtas™ RA in this cohort of biologic naive patients with active RA. This study underscores the fact that given the tremendous burden of RA on its patients and the society, all patients irrespective of sero- positivity or -negativity should be considered for RTX therapy.


  P33 Category: Rheumatoid Arthritis Top


Correlation between Instrumental Hand Function and Activities of Daily Living in Rheumatoid Arthritis

AK Gupta, SL Yadav , U Singh, S Wadhwa, A Kumar, D Borah; All India Institute of Medical Sciences, New Delhi, India

Objective: To find the correlation of instrumental hand function (grip strength, muscle power and range of motion) and Activities of Daily Living (ADL) in rheumatoid arthritis patients.

Methodology: 50 patients of either sex were included in the study. Objective evaluation of hand function was done using Hand dynamometer and Electrogoniometer (Tracker system-version 4®). The patients were assessed for their functional limitations using Indian version of HAQ-DI. Spearman rank collision was performed to find out the association among the variables.

Results: It was found that most of the disease specific parameters like morning stiffness, number of inflamed joints, duration of the disease and deformities had a strong correlation with the instrumental hand function. Deficits in grip strength, tip pinch, palmar pinch, and range of motion of hand strongly correlated to difficulty in activities of daily living in patient with RA. Instrumental hand functions (grip strength, pinch strength and range of motion of joints) were significantly impaired in patient with RA and they had good correlation with Indian Health Assessment Questionnaire Disability Index (IHAQ-DI).

Conclusion: Instrumental hand function assessment along with IHAQ-DI is an effective tool in evaluation and modulation of therapeutic interventions in patients with rheumatoid arthritis. The instrumental hand function assessment can also predict the deficits in ADL.

Key words: Rheumatoid Arthritis, Hand function, Activity of Daily Living.


  P34 Category: Rheumatoid Arthritis Top


Splitting dose of methotrexate in RA management: Making a mountain out of a molehill?

Vinod Ravindran1, 2 ;1 Centre for Rheumatology, Calicut &2 Department of Rheumatology, National Hospital, Calicut

Background: Methotrexate (MTX) is widely regarded as the key to cost effective management of RA and is given orally once weekly. At higher doses however many patients experience troublesome nausea and vomiting resulting in poor treatment adherence. The conventional posology of MTX therefore needs a rethink but the evidence is lacking, probably due to the fear of trying out something new.

Methods:
From the department database all patients who were splitting the dose of MTX over a week (i.e. twice in two different weekdays) were indentified and their diseases related and other relevant parameters were recorded.

Results:
At total of 29 such patients were identified (Table 1). The long term "splitting" was patient initiated and was necessitated by well known adverse effects (AEs) of MTX (i.e. nausea and vomiting) and was facilitated by the lack of availability of single 20 mg or 25 mg of MTX tablets. The efficacy of MTX was maintained and there were no significant AEs (Table 1). In this cohort therefore potential use of glucocorrtiocids was also avoided and by not using the anti-emetics further costs were saved.



Conclusion: Contrary to the popular notion splitting of MTX in patients with RA was safe and without loss of efficacy in this small cohort. This highlights the need for well designed trials looking into this particular aspect.


  P35 Category: Rheumatoid Arthritis Top


Rheumatoid Arthritis causing arthrodesis of atlantoaxial joint presenting as Cervical Myelopathy

1 M V Patel,2 D M Patel; Dhruv healthcare multispecialty Hospital, Smt.NHL municipal medical College, Ahmedabad 380026, Gujarat

Introduction: Cervical complications of rheumatoid arthritis are Atlantoaxial subluxation or cranial odontoid invagination but arthrodesis of atlantoaxial complex causing cervical myelopathy in RA is rare.

Case presentation : A 56 your old female with history of rheumatoid arthritis since last 11 year presented with neck pain, weakened hand-grips and paresthesia in both upper and lower limbs since last 16 months. On examination, she was having active RA with DAS28-CRP score of 5.42 and SDAI score 58.4. Both hands were showing typical RA deformities. On neurological examination, all limbs' power was normal except bilateral mild handgrip weakness. Deep tendon reflexes were exaggerated. Bilateral plantar reflex was extensor. Patient's CRP was 8 mg/l and anti-CCP was 70 IU. X-ray of CV Junction was showing extensive calcification at C1-C2 complex with atlantoaxial subluxation (figure 1). Magnetic resonance imaging (MRI) of CV Junction showed cervical cord compression by posterior arch of C1 along with hypointense lesion around atlantoaxial complex in T1W and T2W sequences suggestive of calcification (figure 2). X-rays of both hands with wrist joints showed evidence of Periarticular osteopenia.



Conclusion: Atlantoaxial subluxation or dislocation in RA usually require atlantoaxial fixation by neurosurgery but in this patient natural arthrodesis of atlantoaxial joint occurred causing anterior displacement of posterior arch of C1 and narrowing of cervical canal which lead to compressive cervical myelopathy.


  P36 Category: Rheumatoid Arthritis Top


RA SLE OVERLAP- RHUPUS - A Case Report

Srikantan Sreedharan Pillai, Sheila Kurian, Rajeevan S; Govt Medical College, Thiruvananthapuram

Abstract: Overlap means combination of more than one rheumatic diseases either clinical or serological. Overlapping features of SLE and RA named as Rhupus is not very common. The estimated prevalence is as low as 0.09% to a maximum of 9.7% as per literature. The prevalence is low in view of the differing pathogenesis. Rhupus with onset of RA typically developed SLE within 7.8 years. Organ involvement particularly renal is not very common in Rhupus. Here we present an interesting case of a 52 year old women diagnosed as RA 17 years back with classical deforming polyarthritis now presenting with features of SLE. She has pancytopenia, low C3, renal involvement(elevated urea and creatinine, P/C ratio =2.3) and ANA, anti-dSDNA and SM positive. Thus this patient has presented with SLE 17 years after diagnosis of RA. In addition she also has renal involvement which is not very common in Rhupus. Usually prognosis in Rhupus is worse than RA but better than SLE




  P37 Category: Polymyalgia Rheumatica Top


A Case Series of Polymyalgia Rheumatica

Joseph T Antony, Jayaraj K, Paul T Antony1 , Geo Paul, T P Antony; Departments of General medicine,1 Clinical Immunology, Amala institute of medical sciences, Thrissur

Background : Polymyalgia rheumatica (PMR) is an age associated, inflammatory musculoskeletal disease with a lifetime risk of 2.4% for women and 1.7% for men, and affects 0.7% of the population over the age of 50 years. There is paucity of clinical data from our part of the world regarding this remarkable disease.

Methods : Consecutive patients attending the general medicine & immunology clinics at amala medical college satisfying the ACR/EULAR classification criteria were enrolled in the study. The clinical details were recorded in a predefined proforma. Detailed laboratory evaluation like CBC, CRP,ESR, LFT, RFT and blood sugars were done in all patients. Autoantibody evaluation was done as per clinical indication to rule out the main differential diagnosis. Ultrasonography of the shoulders and hips as per indication was done by an experienced sonologist.

Results : Fifteen patients were diagnosed to have PMR according to ACR/EULAR criteria. Seventy-five percent of patients (n = 10) were between 55-65 years of age at diagnosis and females were slightly more predominant. The commonest feature at disease onset was bilateral shoulder pain or stiffness (95.5%).Hip stiffness and neck stiffness was present in 70% and 50% respectively. Elevated erythrocyte sedimentation rate >60 mm/h was seen in 100. Ultrasound findings were bilateral only in 80% individuals with bicipital tenosynovitis being the predominant finding. All patients were treated with oral corticosteroids with majority responding at a prednisolone equivalent dose of 15 mg. Methotrexate was the most commonly used steroid sparing agent.

Conclusion : Polymyalgia rheumatic is not an uncommon disease in central Kerala. An expert sonologist can aid in confirming the diagnosis. Treatment response to steroids and methotrexate is good. Long term follow up data needs to be evaluated for outcomes.


  P38 Category: Juvenile Idiopathic Arthritis Top


Systemic onset juvenile idiopathic arthritis: 16 years experience from a single center in India

Pradeepta Sekhar Patro, Ranjan Gupta, Abhishek Zanwar, Able Lawrence, Vikas Agarwal, Ramnath Misra, Amita Aggarwal; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow

Background/Purpose: Systemic onset JIA presents with fever, rash, lymphadenopathy, hepatosplenomegaly and marked rise in inflammatory parameters. It is difficult to diagnose in countries with high infection load. We report our experience of last 16 years in a resource constrained setting.

Methods:
Retrospective case records of all children with SoJI A among 1496 children with JIA seen during the period of 2000-2016 were retrieved. Data on clinical feature, delay in diagnosis, treatment provided and outcome were collected.

Results: There were total of 148 Patients with SoJIA with 102 being boys. Mean age at disease onset and age of presentation was 6.7 yrs (±3.9yrs) and 9.5yrs (±4.9yrs) respectively. At the time of presentation, most common symptoms were fever (99%, n=147), arthritis (95%,n=141), lymphadenopathy(56%,n=83), hepatomegaly(45%,n=66), rash (39%,n= 58) and splenomegaly(34%,n=50). At the time of presentation anemia was present in 70% (99/142) where as leukocytosis and thrombocytosis were observed in 68% (98/143) and 46% (65/140) respectively. Among acute phase reactant raised CRP (>1mg/dl) and ESR (>20mm at 1st hr) were observed in 96%(132/138) and 94% (116/124) respectively. Methotrexate (MTX) (63%) was the most commonly used DMARD along with steroids followed by thalidomide and cyclosporine. The median follow up was 23 months (IQR 7-54 months). Persistent disease (67%) was the most commonly observed course followed by polycyclic (23%) and monocyclic (10%) pattern. Seven patients died, 3 related to MAS, 2 had viral hepatitis; one each had interstitial pneumonia and intestinal obstruction.

Conclusion: Delay in diagnosis is a major problem in SOJIA patients and is associated with a persistent course. Thalidomide and cyclosporine may be viable, alternative steroid-sparing options in patients with MTX-resistant SOJIA in a resource constrained country.


  P39 Category: Juvenile Idiopathic Arthritis Top


Cardiovascular risk assessment in children with Juvenile Idiopathic Arthritis

Karamalla Saddam Hussain, Reena Gulati, Santhosh S1 ,2 VS Negi; Department of Pediatrics,1 Cardiology,2 Clinical Immunology, Jawaharlal institute of postgraduate medical education and research, Pondicherry

Introduction: Diseases with chronic inflammation are known to have higher rates of cardiovascular (CV) morbidity and mortality, e.g. adult rheumatoid arthritis. Juvenile idiopathic arthritis (JIA) patients are exposed to longer duration of chronic inflammation and hence maybe at increased risk of early cardiovascular morbidity. Children at high risk are known to develop atherosclerotic changes as early as first decade of life. Evaluation of risk factors may help in early detection and institution of preventive interventions. We evaluated flow mediated dilatation (FMD) of brachial artery and carotid artery intima-media thickness (CIMT), well accepted surrogate markers of atherosclerotic changes, in young JIA patients.

Materials and Methods: The cross sectional comparative study included 4-18 yr old 42 children with JIA and 42 age and sex matched healthy controls. Baseline demographic data and BMI was recorded. All participants underwent 2D echocardiography for LVMi, and measurement of CIMT and FMD besides serum lipid profiling.

Results: Patients had significantly higher systolic and diastolic blood pressure, total cholesterol, LDL - cholesterol (p <0.001) and lower HDL - cholesterol levels (p <0.001) than controls. The absolute and proportionate changes in FMD were significantly reduced, CIMT and LVMi significantly increased in patients (p = <0.001). CIMT showed positive correlation with SBP (p = 0.001), DBP (p = <0.001), LDL (p = 0.04), disease duration (p = <0.001) and negative correlation with HDL (p = <0.001). Absolute and proportionate change in FMD correlated positively with HDL (p = 0.006, p = 0.012 respectively) and negatively with disease duration (p = <0.001, p = <0.001 respectively). Multiple regression analysis did not reveal any significant predictors for CIMT, absolute or proportionate change in FMD.



Conclusion : Children with JIA have increased LVMi, CIMT, and reduced % of FMD that indicate presence of early cardiovascular dysfunction. Risk reduction intervention may be helpful in preventing CV morbidity later in life.




  P40 Category: Juvenile Idiopathic Arthritis Top


Use of Thalidomide in Systemic Onset Juvenile Idiopathic arthritis (SOJIA)

Manjari Agarwal, Abhay Shivpuri, Sujata Sawhney; Institute of Child Health, Sir Ganga Ram Hospital, New Delhi

Background: Systemic onset Juvenile idiopathic arthritis is the second common subtype of JIA seen in India. A relapsing remitting course is often seen requiring aggressive management with steroids, DMARDs and IL 6 blocker, Tocilizumab. IL6 blockade is beneficial though cost is prohibitive, thus the requirement of another broad spectrum agent. This was undertaken to 1. To study the efficacy of thalidomide in children with SOJIA 2.To study the side effects of thalidomide.

Methods : Retrospective data was studied and clinical details noted of children with SOJIA followed at the pediatric rheumatology unit from 01/06/2009 to 15/08/16. All children fulfilling ILAR classification for SOJIA who received thalidomide during the course of their disease were included.

Results: Thalidomide taken by 46 children.17 girls and 29 boys formed our cohort. Median age at disease onset 4.62 years(0.7-13.5 years),median age at disease diagnosis 5.45 years(1-13.75 years) with 4.5 months median delay to diagnosis. Median age of commencing thalidomide was 6.83 years(1.7-17.25 years). Efficacy: Median dose of oral steroids prior to thalidomide was 0.5mg/kg/day and 6 months after thalidomide was 0.02mg/kg/day, statistically significant (p<0.001). Median dose of thalidomide 3mg/kg/day (1.25-5.7mg/kg/day) as a single bedtime dose to decrease somnolence. Median duration of therapy with thalidomide 20 months(1-64 months).Median time taken for response in systemic features was 1 month(1-18 months). Median swollen joint count prior to thalidomide was 4 and 6 months later was 0 (p<0.05) Median ESR prior to Thalidomide was 66 and at 6 months was 18(p<0.05),median CRP 7.45mg/L prior to Thalidomide and 5mg/L after 6 months. 27 children experienced flares during Thalidomide therapy. Thalidomide stopped in 21(no response: 4,switch over to biologic: 4, remission of disease: 13). Side effects:11 children reported side effects, 2 had severe constipation, 9 had increased somnolence. Conclusion: Thalidomide is a feasible DMARD in lieu of IL6 blockade for this subcategory and has a quick response in systemic features.


  P41 Category: Juvenile Idiopathic Arthritis Top


Lessons learnt from 6 years of biologic use in Juvenile Idiopathic Arthritis at a south Indian tertiary care centre.

Chanakya K1 , Narendra V1 Vara Prasad IR2 , Liza Rajashekar3 ; Nizam's Institute of Medical Sciences.

Aim: To review the registry of patients with JIA receiving biologics for response, dosing schedule and safety

Methods:
All children with JIA receiving biologics were enrolled in biologic register. Demographics, clinical details, dosing schedule, adverse events, steroid and DMARD doses were noted in register. Outcomes assessed as inactive disease and complete remission based on definition by Wallace et al. Descriptive statistics were used to analyze the data.

Results: 20 patients were included. Median age was 4.5 years (range: 2-26), median duration of follow up was 3.75 years (range: 9months-16yrs). Fifteen had SOJIA, 3 had polyarticular JIA, 2 Enthesitis related arthritis (ERA). Seventeen received Tocilizumab, 2 Etanercept and 1 Infliximab. Recommended dosing schedule was followed in 6 patients, others received a lower dosing and a monthly schedule with Tocilizumab. Total of 215 doses of Tocilizumab were given over 19.2 person years. Median time to response was 2 months. Clinical outcomes were as follows: 10 had complete remission on medication, 2 had inactive disease, 2 had some clinical response, 4 had no response. Two patients were lost to follow up. Steroid dose was reduced in all patients. Mean steroid dose at initiation of biologic was 6.75mg (± 3.82) of prednisolone and at last follow up was 4mg (± 1.65). Other DMARDs were stopped in 5, reduced in 1, increased in 5. One patient developed severe macrophage activation syndrome due to delay in procuring tocilizumab. Adverse events were noted in 5 patients. Transaminitis in 3(mild in 2, severe in 1), acute gastroenteritis in 1, neutropenia in 1. On telephonic enquiry on the lost to follow up patients, 2 had died, 1 due to the complication of the disease, possible macrophage activation syndrome and other had a possible infection. 9 were funded by government.

Conclusions: Lower and infrequent dosing schedule with Tocilizumab can still achieve good results in resource constrained setting.


  P42 Category: Juvenile Idiopathic Arthritis Top


Thalidomide in Systemic onset Juvenile Idiopathic Arthritis: Single center experience of 24 patients

Zanwar Abhishek Arvind, Pradipto Patro S, Aggarwal Amita; Sanjay Gandhi Post graduate Institute of Medical Sciences, Lucknow

Introduction: SoJIA treatment has been revolutionized with introduction of IL-6 and IL-1 inhibitors. However in resource constrained situations patients with inadequate response to methotrexate or steroid dependence have very few options. Thalidomide has been reported to be useful in small case series. Thus we did a retrospective review of children treated with thalidomide at our center.

Results: Twenty-four children (18 boys & 6 girls) with SoJIA received thalidomide. The median age was 7 (4-9) years and disease duration was 30 (18-60) months. Fifteen patients had systemic symptoms and 9 had both joint and systemic symptoms at start of therapy. All patients had inadequate disease control with methotrexate. Median hemoglobin was 9.7 gm/dl total leucocyte count was 15200/mm3 platelet count was 320000/mm3 , ESR was 53 mm. Patients received thalidomide for an average of 19.4 (6-24) months. Eighteen patients achieved inactive disease state while 2 patients had partial response and 4 did not respond. Twenty patients had reduction in steroid dose from 0.5mg/kg to 0.1mg/kg and seven patients could get off steroids. Four patients had relapse (2 each on treatment and off treatment with thalidomide). All had inactive disease on subsequent follow up. Thalidomide was well tolerated with only one patient having increased somnolence while none had peripheral neuropathy.

Conclusion: Thalidomide is a cost-effective treatment in SoJIA patients not responding to MTX.


  P43 Category: Juvenile Idiopathic Arthritis Top


"Etanercept In Enthesitis Related Arthritis- Retrospective Study From A Tertiary Care Centre"

Saranya Chinnadurai, Rajeswari Sankaralingam, Tiruchencode Natesan Tamilselvam, Balameena Selvakumar, Ramesh Ramamoorthy, Sowndhariya Velu Annamalai, Sivakumar Vengudusamy; Madras Medical College & Rajiv Gandhi Government General Hospital, Chennai

Background: Enthesitis related arthritis (ERA), a subgroup of juvenile idiopathic arthritis is characterised by arthritis and/or enthesitis. TNF inhibitors are being used in the management of juvenile idiopathic arthritis, unresponsive to NSAIDS and DMARDs.

Objective:
To retrospectively study the outcome of patients with Enthesitis related arthritis, treated with Etanercept for 12 weeks in a tertiary care centre.

Methods: Retrospective study was done in ERA patients (n=23) who received Inj.Etanercept (0.8mg/kg s.c. weekly) for 12 weeks following inadequate response to conventional treatment (NSAIDS & DMARDS).

Results:
Of the 23 ERA patients, 19 (83%) were males and 4 (17%) were females. Mean age of the patients was 15.6+2.31 years. Median disease duration at the time of presentation was 6 months. Etanercept was started in patients after a median duration of 6 months (range 3-18months) of conventional treatment with NSAIDS & DMARDS. 5/23(21.7%) were lost to follow up. 15/18(83.3%) & 11/18 (61.1%) showed inactive disease at the end of 12 & 24 weeks of follow up respectively. The mean number of tender joint count, swollen joint count and tender enthesitis sites decreased by 80%, 82% and 70% respectively at 24 weeks. There is significant reduction in disease activity markers (ESR & CRP) at 24 weeks from baseline (p<0.0001). The mean duration of inactive disease after etanercept therapy was 3 months. Due to persistent disease activity, Etanercept was restarted in 3/18(16.6%). No major side effects were reported in any of these patients.

Conclusion:
Etanercept can used as a safer treatment option for ERA unresponsive to conventional treatment, however long term treatment is required to control the persistent disease activity.

email: saranya_shrishti@yahoo.co.in




  P44 Category: Fibromyalgia Top


Deciphering the level of oxidative and antioxidative parameters and its relationship with clinical symptoms in women with Fibromyalgia Syndrome

Ghizal Fatima1, Siddharth Kumar Das1 , Abbas Ali Mahdi2 ; Department of Rheumatology & Department of Biochemistry, King George's Medical University, Lucknow, India

Background: FMS is a common chronic pain syndrome with an unknown etiology. Increased oxidative stress results from disparity between products of oxidation and antioxidant defenses. There are several clinical conditions integrated with increased oxidative stress, but novel data suggest a relationship between oxidative stress and pain perception. Furthermore, there is scant information available in scientific literature about oxidative and antioxidative parameters in FMS. Therefore in the present study we examined the involvement of oxidative and antioxidative parameters in women with Fibromyalgia Syndrome (FMS) and also evaluated its correlation with the severity of its symptoms.

Methods:
Oxidative stress was determined by measuring the levels of Lipid Peroxides (LPO), nitric oxide (NO) and Protein carbonyl in plasma and antioxidative parameters like catalase, Glutathione peroxidase (GPx) and Glutathione Reductase (GR) in lysate in 60 female patients satisfying American College of Rheumatology (ACR) criteria for FMS and 60 healthy females without FMS. Clinical parameters of FMS were evaluated by Fibromyalgia Impact Questionnaire Revised (FIQR).

Results:
Concentrations of catalase (p<0.01), GR (p<0.01) and GPx (p<0.01) were significantly lower in patients with FMS than in controls, and levels of oxidative stress parameters, LPO (p<0.01), NO (p<0.01) and Protein carbonyl (p<0.01) were significantly higher in patients than in controls. A significant positive correlation was found between LPO and clinical symptoms of FMS among patients group. Furthermore, a significant positive correlation was also found between Protein carbonyl and clinical symptoms of FMS among patients group than in control group.

Conclusion:
The present results indicate that women with FMS are exposed to oxidative stress and this escalated oxidative stress may play a role in the etiopathogenesis of the disease. Moreover, our results also show that increased oxidative stress parameters are more strongly amalgamated with FMS symptoms.


  P45 Category: Fibromyalgia Top


Cross sectional study of various psychosocial variables in Fibromyalgia patients in tertiary health care setting.

Srinivasa C, Tiwary N, Shantha M, Natasha, Shailee C, Singhai S, Dharmanand BG; Sakra World hospital, Bengaluru, Karnataka

Introduction: Fibromyalgia (FM) is one of the common diagnosis made in rheumatology settings. India. Better understanding of contributing social and psychological variables will certainly enhance clinical understanding and patient care. This study targets various cognitive and quality of life related variables.

Material and Methods: Stratified random sample of 87 patients, from Rheumatology clinic, Sakra World Hospital, Bangalore, May 2014 to Feb 2015, diagnosed as FM by rheumatologist were included in the study. Visual Analogue Scale for pain (VAS), Fibromyalgia Impact Questionnaire (FIQ), Brief Pain Inventory (BPI), Hospital Anxiety and Depression Scale (HADS), Pain Catastrophization Scale (PCS), Self-efficacy Scale (SES), WHO- Quality of Life Scale (Brief) were applied.

Results:
92% were females and 8 % males. Mean age was 41.55 (10.5). Mean VAS score was 5.18 (2.17). 51(64%) had VAS more than 5. Mean FIQ score was 47.41(18.18) which falls in moderate category, 23(26%) patients had very high FIQ SCORES. Mean Scores for BPI (severity) was 4.72 (1.97) and BPI (interference) was 4.63(2.35), both falls in moderate severity category. 10(11.4%) had high BPI PAIN SEVERITY SCALE. 14 (16%) patients had high BPI- INTERFERANCE SCALE. On HADS, Mean Anxiety was 9.60 (5.08) and Mean Depression was 8.36(4.89) and both were clinically significant. PCS had mean score of 26 (12.56) which indicated significant catastrophization. 33(37%) had significant PCS SCORES. SES mean score was 49.95 (14.80) highest score on efficacy being 80. 26 (29%) had low SES scale. WHO-QOL (Brief) mean and SD scores were as follows. Physical domain Mean 82.91(17.49), Psychological domain mean 76.28(16.83), Social Domain mean 43.54 (8.17), Environmental domain mean 120.8(18.88).

Conclusion: Pain severity, interference, depression, anxiety, catastrophization of pain and overall impact of fibromyalgia was significantly higher in more than 50% of all patients. Usage of these scales would identify the domains which needs attention during formulating management plan.


  P46 Category: Fibromyalgia Top


Myofascial pain Trigger points: A frequently overlooked pain generator, in established rheumatic painful diseases.

Sudheernath Sake, Dharmanand B.G; Sakra world Hospital, Bangalore

Background: Myofascial syndrome (MFPS) is one of the common causes for musculoskeletal pains. Trigger point (TP) examination is not routinely done during clinical visits for inflammatory arthritis. Thus it may be overlooked as a cause for pain in these conditions and pain may be attributed to the disease and treatment escalation may be done with its risks. We present three cases where treatments of TP (TPM) lead to significant improvement.

Case 1: 53 year old female with Ankylosing Spondylitis (AS), presented with neck pain, frequent headaches and pain radiating to upper limbs. There was no relief with NSAIDs. She was found to have TP in Trapezius and Sternocleidomastoid. Patient was treated for 10 sessions with Trigger point massage, stretching exercises and dry needling. Pain reduced by 80% along with headaches and limb pain.

Case 2: 43 year old female with AS of more than 10 years duration presented with increased mid and low back pain. Examination revealed significant stiffness and reduced ROM of spine on muscle palpation she had numerous trigger points in the para spinal muscles. The patient was treated with Trigger point release technique, Ultrasound massage, Cryotherapy and dry needling along with good stretching exercises. Clinically significant relief was seen with 80% reduction in intensity pain intensity

Case 3: 46 year old female with Sjogren's syndrome who underwent THR for AVN of left hip one year ago presented with severe hip pain and difficulty to walk. Imaging did not reveal and bony or peri-prosthetic lesions. She was found to have TP in quadriceps lateralis, Gluteus medius and adductors. TPM including dry needling gave her complete relief.

Conclusion: It is essential to look for pain generators even in established rheumatic diseases. MFPS is a treatable condition using trigger point management


  P47 Category: Crystal Arthritis Top


Ultrasound and Urate crystal deposition: How many joints to screen?

Danveer Bhadu (MD)1 , Siddharth K Das (MD)1 , Archana Wakhlu (MBBS, DMRD)1 , Anupam Wakhlu (MD, DM)1 , Urmila Dhakad (MD)1 , Puneet Kumar (MD)1 , Meha Sharma (MD)1 ; King George's Medical University, Lucknow, India

Objective: To determine the diagnostic value of combinations of intra-articular and periarticular ultrasound-detected abnormalities namely double contour sign (DCS) and hyperechoic aggregates (HAGs) in the upper and lower limbs in patients of gout and asymptomatic hyperuricemia (AH).

Methods: Forty seven gout, twenty four AH (SUA ≥7mg/dl) and forty controls (SUA <7mg/dl) with age more than 18 years were included in this study. DCS was looked for at three articular cartilage sites (first metatarsal, tibiotalar and femoral condyle) whereas HAGs were looked for at one joint site (radiocarpal joint) and two tendon sites (patellar tendon and triceps tendon). Ultrasound findings of all three groups were compared.

Results:
We found sensitivity, specificity, positive predictive value, negative predictive value and positive likelihood ratio of two joint areas (knee and 1st metatarsophalangeal (MTP)) ultrasound findings for gout were 87.2%, 82.5%, 85.4%, 86.4% and 5.0 respectively. Similar Sensitivity, specificity, positive predictive value, negative predictive value and positive likelihood ratio were observed with global ultrasound findings. The detection rate of ultrasound abnormalities in AH patients was 45.8% with two joint area (knee and 1st MTP) and 50% with global. Amongst controls 17.5% were found to have these abnormal ultrasound findings by both two joint area and global exams.

Conclusion:
Screening of two joint area (knee and 1st MTP) has similar sensitivity, specificity and positive likelihood ratio as compared to global (six sites) in diagnosing gout and has good detection rate of urate crystal deposition in AH patients.


  P48 Category: Crystal Arthritis Top


Allopurinol reduces the risk of Myocardial Infarction (MI) in the Elderly: A Study of Medicare Claims

Jasvinder A. Singh, MBBS, MPH1, 2, 3 , Shaohua Yu, MS2 ; Birmingham VA Medical Center, Birmingham, AL,2 Department of Medicine at School of Medicine, and Division of Epidemiology at School of Public Health, University of Alabama at Birmingham (UAB), Birmingham, AL;3 Department of Orthopedic Surgery, Mayo Clinic College of Medicine, Rochester, MN.

Background : Previous observational studies that examined the association of allopurinol with myocardial infarction (MI) provided contradictory results. One study showed that allopurinol reduced the risk and the other study showed an increased risk with allopurinol. Therefore, our objective was to assess whether allopurinol use is associated with a reduction in the risk of MI in the elderly.

Method : We used the 2006-2012 5% random sample of Medicare beneficiaries to study the association of new allopurinol initiation and the risk of incident MI, in a cohort study. Multivariable-adjusted Cox regression models adjusted for age, gender, race and Charlson index, in addition to various cardio-protective medications (beta-blockers, ACE inhibitors, diuretics, statins). We calculated hazards ratio (HR) with 95% confidence intervals (CI). Sensitivity analyses adjusted for coronary artery disease (CAD) risk factors including hypertension, hyperlipidemia, diabetes, and smoking.

Results : 1,544 of the 29,298 episodes of incident allopurinol use were associated with incident MI (5.3% episodes). Allopurinol use was associated with reduced hazards of MI, with a HR of 0.85 (95% CI, 0.77 to 0.95). Compared to no allopurinol use, longer durations of allopurinol use were associated with a lower HR of MI: 1-180 days, 0.98 (95% CI, 0.84 to 1.14); 181 days to 2 years, 0.83 (95% CI, 0.72 to 0.95); and >2 years, 0.70 (95% CI, 0.56 to 0.88). Other factors associated with a higher hazard of MI were: age 75-<85 and ≥85, male gender, higher Charlson index score and the use of an ACE-inhibitor. Adjustment for CAD risk factors confirmed these findings.

Conclusion : Incident allopurinol use was associated with a reduction in the risk of incident MI in the elderly. Longer durations of allopurinol use reduced the risk of incident MI incrementally. Future studies should assess the underlying mechanisms for MI prevention and assess the risk-benefit ratio for allopurinol use.


  P49 Category: Crystal Arthritis Top


An Atypical Experience with Gout

Nachiket Kulkarni, Kiranjit Singh; Jehangir Hospital, Pune

Background : Gout is known to mankind since ages but still baffles a Rheumatologist in atypical presentations.

Methods : Case scenarios identified from single centre database of hospital based setting from out and in patient lists. The selection was random based on merit of the case. Gout was classified based on 2015 ACR/EULAR Classification criteria. Clinical and laboratory details were recovered.

Results : This series highlights three case scenarios. Case 1: 55 year gentlemen with acute polyarthritis and fever (simultaneous onset) of 5 days. Comorbidities of Diabetes, Hypertension on treatment. Laboratory: polymorphonuclear leucocytosis, nephropathy, microalbuminuria. Infectious diseases screened and tests negative. Rheumatoid factor high positive, ACCP and ANA negative. In view of therapeutic dilemma; right knee fluid aspiration was done which showed uric acid crystals. Serum uric acid(SUA) levels were 13.1mg/dl. Initiated on moderate dose steroids gradually tapered and addition of Febuxostat. Case 2: 65 year lady with Osteoarthritis of knee, Diabetes Mellitus and Hypertension. Presented with Septic shock due to urinary tract infection. Second day she developed both knee synovitis. The knee fluid tapped. Features -exudative, Uric acid crystals noted, sterile. SUA 9.5mg/dl. Low dose steroids and Febuxostat added to ongoing treatment. Case 3: 31 year gentlemen. No comorbidities. Had an acute(3 days) left tibialis anterior enthesopathy. Targeted sonography showed enthesial inflammation and soft to firm consistency nodule. Laboratory- leucocytosis, hyperuricemia(9.1mg/dl). Sonography of abdomen showed renal calculi. A needle aspiration of the nodule was done. Uric acid crystals evident on aspirate. He was initiated on NSAIDS and based on clinical discretion Allopurinol initiated. Outcome favourable in all cases.

Conclusion : Atypical presentations(Polyarticular, prior diseased joint involvement, enthesopathy) of Gout need attention. Gout stays a differential in varied presentation of Inflammatory Arthritis.


  P50 Category: Biologics Top


Etanercept In Uveitis : Ineffective Or Unsafe ? Anterior Uveitis In Spondyloarthropathy Treated With Etanercept

Balaji Chilukuri, Rajeswari Sankaralingam, Ramesh Ramamoorthy, Tiruchengode Natesan Tamilselvam, Bhuvanesh Mahendran, Saranya Chinnadurai, Sivakumar Vengudusamy, Mythili Seetharaman; Institute of Rheumatology, Madras Medical College

Background:
Anti-tumour necrosis factor (TNF) agents are being increasingly used in treatment of both peripheral and axial spondyloarthropathy (SpA).Uveitis is the most common extra articular manifestation of SpA. The use of anti-TNF therapies in patients with SpA has been associated with a reduction in uveitis flares.However paradoxical new onset ocular inflammation has been reported after initiation of anti-TNF agents.

Method:
65 patients of spondyloarthropathy on anti-TNF agents from a cohort of 237 cases were included in this study.43 cases received etanercept 50 mg sc weekly,22 cases received infliximab 3 mg/kg monthly following a loading dose. Demographic,clinical and laboratory parameters were recorded. Baseline ophthalmological screening for uveitis was done for all patients and they were followed up periodically. Only patients without baseline uveitis were included in the study.

Results :
Baseline characteristics were comparable in both groups. 5 cases (11.6%) on etanercept therapy developed acute anterior uveitis (AAU) whereas none of the patients on infliximab developed ocular complications includind uveitis.Four out of five patients were HLA B27 positive.Mean BASDAI in patients who developed uveitis was 6.3 indicating higher disease activity.Mean duration of onset of uveitis after starting etanercept therapy was 7±3 days.Two out of five patients developed glaucoma. One patient with psoriatic arthritis developed exacerbations of psoriatic skin lesions and new biopsy proven vasculitic skin lesions.Uveitis and disease activity improved after switching over these patients to infliximab therapy.There was no relapse of uveitis in any of the five patients treated with infliximab after a follow up of one year.

Conclusion :
We observed that use of anti-TNF fusion protein 'Etanercept' is associated with increased incidence of new onset AAU in SpA. Monoclonal anti-TNF 'Infliximab' was not associated with new onset AAU in this study.


  P51 Category:Biologics Top


Tuberculosis profile in Biologics experienced patients- Karnataka Biologics Cohort (KBC) Study Group experience

Vineeta Shobha, Vijay Rao, Anu Desai, Ramesh Jois, Chandrashekara Srikantiah, Dharmanand B.G, Sharath Kumar, Pradeep Kumar, Chethana Dharmapalaiah, Mahendranath KM, Shiva Prasad, Manisha Daware, Yogesh Singh, Uma Karjigi; St John's Medical College Hospital, Manipal Hospital, Fortis Hospital, Chanre Rheumatology and ImmunologyResearch Centre, Sakra Hospital, Columbai Asia Hospital, Apollo hospital, Samarpan Health Centre, Vikram Hospital and Heart Care, Mysore, Narayana Hrudalaya

Introduction : Safety of biologics response modifiers is a matter of concern especially in immunosuppressed patients in high burden regions like India. TB risk in Indian patients on biologics has not been precisely evaluated to date. To fill up the lacunae in the awareness of safe biologics usage, current study is undertaken in setting of day today clinical practice in Karnataka state, India.

Materials and Methods : This is a cross sectional observational study conducted across 10 centres located in Karnataka, India and included 411 biologics experienced patients over last 24 months. All subjects underwent screening for latent tuberculosis which included tuberculin test or TB quantiferon or both. Chest X Ray was performed in all patients; HRCT chest was performed if there was high index of suspicion of pulmonary tuberculosis (n=23).

Results: Subjects were exposed to entire gamut of Biologics available in Indian market (TNF#=255, Rituximab=113, Tociluzumab=39, Abatacept=4) for various autoimmune Indications, average duration of use being 11.64 months per patient. 43/411(10.46%) cases with evidence of latent tuberculosis were offered prophylactic therapy and then treated with biologics. 10/411 (2.4%) cases suffered tuberculosis at varying time intervals, these included pulm/ extrapulm/ disseminated (3/3/4). However, all these cases had tested negative on pre-screening investigations for TB. Despite known limitations of tuberculosis pre screening methods, this might suggests that tuberculosis developed in these patients due to new exposure rather than reactivation of latent TB. 8/10 tuberculosis patients responded well to ATT, one developed meningeal TB, another one succumbed to disseminated TB. All of these were on TNF inhibitors, 7 being infliximab or its biosimilar. Also, none administered prophylactic ATT developed tuberculosis post exposure to biologics.

Conclusion : This reconfirms usefulness of prescreening methods in risk stratification of subjects at greater risk for developing tuberculosis and efficacy of antituberosis prophylaxis regimens being used in everyday clinical practice.


  P52 Category: Biologics Top


Safety of Rituximab in an Indian Context

Bimlesh Dhar Pandey, Jinisha Shukla, Molly Thabbah; Department of Rheumatology, Fortis Hospital, Noida, JIPMER, Pondicherry

Background: The safety of biologicals should be tailored for a geographical context and a western guideline (ACR/EULAR guideline) need not be adhered for counties with a high incidence of tuberculosis and infectious disease.

Objectives: To evaluate incidence of tuberculosis and other infections on patients on Rituximab for various autoimmune disorders.

Methods: This was a retrospective observational single center study in which we analyzed records of the patients from 2010-2015 who were on Rituximab. Demographic data, dose and frequency of Rituximab administered, adverse events and episodes of infections were recorded.

Results: Of the total of 238 patients who were on different biologicals, 85(35.7%) were on Rituximab for various autoimmune disorders, RA 67(78.82%), Scleroderma 6(7.06%), SLE 1(1.18%), Sjogren's Syndrome 4(4.71%), Dermatomyositis 2(2.35%), GPA (ANCA associated vasculitis) 2(2.23%), MCTD Type 1(1.18%), Pemphigus Vulgaris 3(3.53%). Mean±SD age of the patients was 52.13±14.63 (16.28% males). 3(3.53%) had infections during the course of treatment. Adverse events were recorded in 6(7.06%) patients and were related to abdominal pain, vomiting and diarrhea (3), rashes (2), Upper GI bleed (1). Incidence of tuberculosis during the treatment regimen was nil, however, there was one patient with multiple Pott's spine and aggressive RA was successfully treated with ATT and Rituximab and DMARDs. 1 patient of RA died of exacerbation of ILD, 1 patient of Scleroderma with Arthritis succumbed to aspiration pneumonia unrelated to the drug and disease.

Conclusion: Rituximab is a safe biological agent for various connective tissues and autoimmune disorder especially for country like India which has a high prevalence of tuberculosis


  P53 Category: Biologics Top


Reduced injection site pain with succinate buffer based Adalimumab biosimilar injection (SUFFER Study): An observational study

Mihir Gharia, Sudhakar A; Zydus Biovation, Zydus Cadila Healthcare Ltd.

Background/Purpose: Pain due to subcutaneous injections often leads to patient incompliance. Since citrate buffer based formulations have been associated with pain and discomfort at injection site1, hence we wanted to evaluate the pain associated with adalimumab biosimilar (Exemptia™), which is formulated using succinate buffer.

Methods: We conducted a prospective questionnaire based study in patients treated with adalimumab (Exemptia™) injection for rheumatological conditions. Patients aged 18 & above of either sex suffering from rheumatoid arthritis (RA), ankylosing spondylitis (AS) and psoriatic arthritis (PsA) were included. We collected data (N=500) with respect to pain at the site of injection with the aid of a questionnaire over 6 months and had completed at least 3 doses of injection. Primary objective is pain at the site within 24 h of injection. Pain was assessed using the verbal pain intensity scale (National Initiative on Pain Control™).

Results: Patients in this study were aged between 18 and 65 years (mean 40.61 ±13.87 years); with Male=357 and Females=119. We assessed the pain following the use of succinate buffer injection of adalimumab injection in 476 (24 excluded) patients with rheumatoid arthritis (RA; N=93), ankylosing spondylitis (AS; N=360) and Psoriatic arthritis (N=23). Majority of the patients (83.12%) reported 'no pain' (Figure 1). On the verbal pain intensity scale, more than 90% of the patients reported 'mild pain' or less. None of the patients reported 'extreme pain' or 'worst possible pain'. One patient with ankylosing spondylitis reported 'severe pain' (Figure 1).



Conclusion: In our study, use of succinate buffer adalimumab (Exemptia™) injection was associated with no pain or mild pain. The outcome of this pilot study needs to be confirmed with a larger control-group trial.


  P54 Category: Biologics Top


Prescribing patterns and safety of Biologics & Biosimilars in Immune Mediated Rheumatic Diseases: Karnataka Biologics Cohort (KBC) Study Group experience

Vineeta Shobha, Vijay Rao, Anu Desai, Ramesh Jois, Chandrashekara Srikantiah, Dharmanand B.G, Sharath Kumar, Pradeep Kumar, Chethana Dharmapalaiah, Mahendranath KM, Shiva Prasad, Manisha Daware, Yogesh Singh, Uma Karjigi; St John's Medical College Hospital, Manipal Hospital, Fortis Hospital, Chanre Rheumatology and Immunology Research Centre, Sakra Hospital, Columbai Asia Hospital, Apollo hospital, Samarpan Health Centre, Vikram Hospital and Heart Care, Mysore, Narayana Hrudalaya.

Introduction: Biologics have stood the test of time nearing 2 decades, but there is a lack of formal data collection which systematically captures prescribing pattern and choice in real life clinical practice. This study is first of its kind approach that attempts to highlight the usage and safety profile of both biologics and biosimilars in India.

Material and Methods:
This is a cross-sectional retrospective study, conducted at ten tertiary care rheumatology centres in Karnataka, India using a comprehensive prestuctured Proforma. Patients suffering from rheumatoid arthritis, spondylo-arthropathies like ankylosing spondylitis, psoriatic arthritis, SLE, vasculitis and others are treated with combination of conventional DMARDs as the first line of management. Patients with aggressive disease who had poor relief to DMARDs/ immunosuppressants are advocated Biologic therapy (TNF blockers, IL-6 antagonists, Abatacept, Rituximab and their biosimilars) and their biosimilars. 411 such biologics experienced patients over last 2 years were included in this study. Inclusion criteria included use of any biologic for any rheumatologic indication. Their baseline characteristics, prescribing pattern, duration of prescription, and adverse effects were studied.

Results:




Conclusions : This is the largest collaborative cohort of Biologics experience from India. Highlight of this study is its unique design in setting of day today clinical practice. Expectedly, TNF# are most frequently used biologics. Despite diverse medical setups catering to varied patient populations, prescribing patterns and safety is similar across centres.


  P55 Category: Biologics Top


Use of Biologics/Biosimilars in Gujarat: An Audit

Taral Parikh, Sapan Pandya, Vishnu Sharma, Puja Srivastava, Manish Bavalia, Rakesh Tank, Rakesh Solanki, Shabbir Chikkani; Rheumatology Association of Gujarat (RAG), Ahmadabad, Gujarat

Background/Purpose: Biologics have been used in rheumatologic diseases since more than a decade. With the advent of biosimilars; affordability and thus use of biologics has increased. Biologics registries give a real life experience of these agents. This is an effort to study the biologic use in clinical practice in Gujarat.

Methods:
Data was collected in a proforma from all centres from Gujarat. Data for 87 patients was analysed (from July 2015 to September 2016). Indication for use, screening, vaccination, adverse events and outcomes were analysed.

Results:
Demographic profile: (n=87) Mean age: 42.72 (± 16.15) years. Mean duration of illness: 8.06 (± 6.12) (6 months-25 years). (F/M) 52/35. (1.48). 75 patients- self payers, 12 - sponsorship.

Indications for use : RA 32 (ILD 4), AS / SpA 21 (IBD 1, amyloid 1, Hep B 1), Sjogren's 7 (lymphoma 1, RTA 1), SLE 7(APLA 3), JIA 5 (ERA 4; polyarticular 1), AOSD and GPA 3, PsA and Pm Scl 2, 1 each MCTD, DM, AIHA, EGPA, mononueritis multiplex. Used: remission induction -46 (just started on biologics)/ induction and long term maintenance- 41. Indication of use was active disease despite conventional treatment in 84, 1 patient treated for amyloid(AS), 1 had loss of response to ongoing TNFi (SpA), 1 patient (RA) had multiple biologic failures.

Screening for infections was done in 50; vaccination was given to 6 patients.



Response 56 (64%) improved, 2 non response, 1 death, 28 very early to assess response

Conclusion: Most common indication was RA followed by SpA, disease refractory to conventional treatment was the most common indication for use and biosimilars were prescribed more than originators. 64% had response to treatment and 12.5% had adverse events. Screening for infection should be implemented more stringently and vaccination still remains an ignored issue.


  P56 Category: Biologics Top


Effects of Teriparatide - does setting influence outcome?

Sham S1 , Pande I2 ; Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham, UK

Introduction: The aim of this study was to assess whether the response to Teriparatide used in a clinic setting was of the same magnitude as in controlled clinical trials.

Materials and Methodology: The rheumatology department offers a fortnightly bone health clinic using standard operating procedure to ensure all patients referred to this clinic have a "one stop" experience. Standardised questionnaires capture risk factors, standard blood screen on early morning fasting sample ensures correct interpretation of bone markers (BMs) and sex hormones in men. Standard drug monitoring protocols for each bone agent means all patients on Teriparatide have DEXA at baseline and 2 years, BMs and vitamin D at periodic intervals.

Results: Teriparatide had been prescribed in 38 patients (26 females, 12 males; mean age 69.1 (±12.5) years; age range 30-88). Majority (27) had severe osteoporosis with multiple vertebral fractures with 3 or more co-morbidities in 20. Majority of patients (28) had oral bisphosphonates before Teriparatide. It was used as a first line therapy in only 8 patients, as 2nd line in 23 patients, as 3rd line in 5 and as 4th line in 2 patients. Eighteen patients completed treatment, 13 are currently on, 4 died and 3 had adverse events. After completing therapy, zoledronate was used in majority (7). Table below shows the BMD and BMs data. In the median follow up of 41.5 (range 3 -116) months, there was only 1 incident fracture.

Conclusion : In contrast to trials, patients in routine clinics requiring Teriparatide are complex, have multiple co-morbidities and majority have had prior treatment with other bone agents. Although response to therapy as a group is not dissimilar at an individual patient level there are wide variations in direction and level of response. Despite the heterogeneity in individual responses re-fracture rate as a cohort is impressive.




  P57 Category: Miscellaneous Top


Listeriosis in Rheumatology a Rare Scare



Nachiket Kulkarni, Arvind Chopra; Centre For Rheumatic Diseases, Pune

Background : Listeriosis has been reported as a rare infection in Rheumatological setting. This series expands the protean manifestations of such occurence.

Methods : Single centre retrospective data collection. Patients identified from referral database maintained at CRD, Pune since 1997 with over 60000 patients. Base line Rheumatological diagnosis was clinical. Listeriosis diagnosed on culture(Listeria Monocytogen -LM) of infected tissue. Clinical and laboratory profile of the cases retrieved in detail. Study period : Jan 2015-July 2016.

Results : Listeriosis identified in 3 cases. Case 1: 73yr lady with chronic deforming Rheumatoid arthritis & Hypertension. On suboptimal irregular DMARDS. Bilateral Knee replacements done. Presented with meningitis. Culture revealed LM. Recieved Ampicillin. Discharged 2nd week. Readmitted after 1 month for Left knee swelling. Aspiration -Pus & Culture demonstrated LM. Revision TKR with 6 week ampicillin administered. Favourable outcome. Case2: 52 year lady referred with PUO and worsening nephropathy. AAV(Anti PR3) with RPGN identified. During hospitalisation she rapidly worsened with features of ARDS, myocarditis, CNS vasculitis, neuropathy. She responded to Plasma pheresis which was followed by two infusions of 1gm Rituximab over fortnight. A repeat admission after 10 days of 2nd infusion was noted for meningitis. IVIg and broad spectrum antibiotics initiated. LM identifieded on culture of CSF and blood. Antibiotics tailored accordingly. Patient succumbed by 2nd week to septic shock. Case 3: 40 yr gentlemen with Chronic SLE. Dominant features over 10 Year course were Inflammatory Arthritis, Lupus Nephritis, Cutaneous manifestation, Anti dsDNA Positivity. He was on regular follow up. Presented with seizure and delirium, concurrent SLE features were quiescent. MRI Brain identified ring enhancing lesions. Brain biopsy of the lesions done in view of atypical site and no evidence of any specific organism available. The tissue on culture identified LM. Complete recovery on initiating ampicillin.

Conclusion : Listeriosis is a rare occurrence in Rheumatological setting. Manifestations are protean with grave presentation. Mortalities noted.


  P58 Category: Lab Immunology Top


Utility of Antibodies to Phosphatidylserine/Prothrombin Complex in Indian Patients

Arvind G1 , Ruchika Goel1 , Jayakanthan K1 , Mahasampath Gowri2 , John Mathew1 , Debashish Danda1; Department of Rheumatology & Department of Biostatistics, Christian Medical College, Vellore

Background: To explore utility of Antibodies to Phosphatidylserine/ Prothrombin Complex (aPSPT), in Indian Anti phospholipid Syndrome (APS) patients.

Methods : Data of 372 subjects whose sera was tested for aPSPT by commercial ELISA (AESUKU,GERMANY) at our centre, was retrospectively analysed. Performance of various APLs was compared using nonparametric tests. To ascertain additional utility of aPSPT, patients with suspected APS were categorized into 2 groups i.e. i) those with any one of criteria antibodies positive ii) those with any one APL positivity including anti- PSPT positivity.

Results: Of 372 subjects (age: 32± 10 years, 89% females) tested for aPSPT, 46 patients satisfied revised Sapporo criteria for APS (20 primary,26 secondary). Remaining subjects were either healthy/diseased controls including 60 patients with seronegative APS (SNAPS). Clinical manifestations in APS patients comprised of 38/46 (82.60%) with thrombotic events (21 arterial, 26 venous) and 11/46(23.91%) with obstetric events. aPSPT tested positive for 41 (11%) of total subjects, of these 9 were SNAPS. Among definite APS patients, Lupus anticoagulant was present in 37/46 (80.43%) followed by anticardiolipin antibody in 19/46 (41.33 %), aPSPT in 17/46 (36.95%), anti-β2glycoprotein1 (β2GP1) IgG and IgM in 13/46 (28.26%) and 7/46 (15.21%) patients respectively. aPSPT did not differ from other criteria APLs in any clinical parameter except compared to those with IgM B2GP-1 positivity. aPSPT was numerically more sensitive and specific than IgM B2GP1 for thrombotic, non-criteria events but less sensitive for detecting obstetric events (Table). Inclusion of aPSPT test in patients with suspected APS did increase sensitivity marginally but with concomitant decrease in specificity.

Conclusion : aPSPT was third most common antibody present in our cohort of APS patients. aPSPT had higher sensitivity and specificity in comparison to IgM anti-β2 GP1 for all APS manifestations except obstetric ones. Seronegative APS patients may test positive for aPSPT, thus endorsing its inclusion in classification criteria of APS.




  P59 Category: Lab Immunology Top


Comparison of Performance of Indirect Immunofluorescence with Immunoblot for The Testing of Anti Nuclear Antibodies

Jairaj Euphrasia Latha, Rajeswari Sankaralingam, Therese Mary Dhason, Balaji Chilukuri, Bhuvanesh Mahendran, Kumudha Manoharan, Mohanraj G; Institute of Rheumatology, Madras Medical College, Chennai

Background: A large number of patients suspected to have a connective tissue disorder are ordered Antinuclear antibodies (ANA) by 'Indirect Immunofluorescence' (IIF) and 'Immunoblot' simultaneously.ANA by IIF is a sensitive assay and immunoblot is a specific assay. ANA related tests are one of the most expensive tests ordered by Physicians and Rheumatologists.Is there a need to order both ANA by IIF and immunoblot simultaneously for all patients ?

Aim:
To compare the performance of IIF with Immunoblot and find out the correlation of patterns detected in IIF with the autoantibodies detected in Immunoblot.

Methods:
400 consecutive patients with clinical suspicion of connective tissue disorder were included in this study.They were tested for ANAby IIF (Hep 2000) 1 in 80 dilution and immunoblot (ANA Profile 3) simultaneously

Results:




Conclusion: ANA Patterns detected by IIF highly correlated with the autoantibodies detected in Immunoblot in this study.Indirect Immunofluorescence can effectively be used as a cost effective, sensitive test for ANA detection.Immunoblot can be reserved for solving clinical dilemmas and research purposes.


  P60 Category: Lab Immunology Top


Prevalence of autoimmune markers in the normal adult population residing in a metropolitan city of India

Kanjilal Maumita,1 Kumar Uma2 Das Nibhriti,3 Soni Vijay,1 Dasgupta Sauvik;1 Department of Rheumatology,2 Department of Biochemistry, All India Institute of Medical Sciences, New Delhi, India,3 Environment Monitoring and Research Center, Indian Metrological Department, New Delhi, India

Background: Over the last few decades prevalence of autoimmune diseases has increased by more than two folds highlighting the role of environmental factors in autoimmunity. This study was planned to study the prevalence of autoantibodies and inflammatory markers in normal healthy subjects residing in Delhi for more than 10 years.

Methods: 300 healthy subjects ≥18 years of age were randomly chosen from five zones of Delhi and their samples were analyzed for presence of RF,ANA, ANCA, Anti CCP, TNF α, IL6, IL17,IL 1β, and hsCRP. The distance from the subject's residence to main road was calculated by using the Google mapping system, which was used as a proxy for traffic pollution exposure.

Results: Mean age of study subjects was 31.0 ± 8.3yrs with 83% (413/500) being females. Frequency of occurrence of autoantibody & inflammatory markers is shown in table 1. It was observed that the subjects residing within 200 meter distance from the main road had higher prevalence of ANA (12.5 % vs 6.3 % ; p value = 0.0260 OR= 2.053; RR= 1.921; CI 1.103 to 3.818) and RF (9.6 % vs 4.3 %; p value =0.0277 OR 2.283 RR 2.1 ;CI 1.109 to 4.702) as compared to the subject residing at or more than 200 meters away from the main road. The Anti CCP and other auto immune markers were not found significantly different in two groups (people residing within 200meters from main road and those beyond that) It was also observed that the inflammatory markers TNF α (11.5 % vs 5.4 % p value = 0.0185; RR 2.106; OR= 2.106 CI =1.163 to 4.352) IL 17 A (11.0 % vs 6.5 % p value = 0.0273, RR =2.018, OR =2.145) hs CRP 12.5 % vs 6.1% (p value = 0.0162, RR = 2.028, OR =2.175 CI ="1.159 to 4.082") differed significantly in two groups.



Conclusion: The prevalence of the autoimmune markers and inflammatory markers was high in subjects residing within 200 meters from the main road where the air pollution is higher. It points towards the plausible role of air pollution in autoimmunity.


  P61 Category: Miscellaneous Top


Vaccinaton Compliance among Patients in Rheumatology Clinic

Thakran R1 , Baghel S S2 , Zaheer Q3 , Kapoor S4 , Garg SR4 , Vivekanand5 , Rawat R2 , Messi C1 , Patil N S6 , Aggarwal N6 , Sharma P6 , Negi H7 , Malaviya A N8 ; Indian Spinal Injuries Centre, New Delhi 110070

Background: The Rheumatology patients with autoimmune inflammatory diseases have increased burden of infectious disease-related morbidity and mortality and have twice the risk of severe infection as compared to the general population.This may be due to disease itself, shared risk factors such as smoking, comorbidities and immunosuppressive treatment.

Purpose: The purpose of this study was to assess the vaccination compliance among patients with rheumatic diseases.

Methods: A survey was conducted to check compliance with vaccination status among 160 patients [123 (76.87%) females and 37 (23.12%) males-{Female: Male [3:3:1]} attending a busy rheumatology clinic.

Results: Total number of patients: 160 patients advised vaccination on their first visit to rheumatology clinic. Patient's diagnosis as follows: Rheumatoid Arthritis (RA): 118 (73.75%), Spondyloarthropathy (SPA): 21 (13.125%) Other rheumatic disease: (MCTD, SLE, Myositis, Dermatomyositis etc): 21(13.125%)





Conclusion:
Patients with rheumatic diseases have high compliance to the advised vaccinations. Strict recommendation by consulting rheumatologist and intense counselling by specialist nurse could be the likely reasons for good compliance to vaccination.


  P62 Category: Miscellaneous Top


Systemic association and outcome with immunosuppressants : An experience from private rheumatology clinic and a tertiary center in Mumbai

Dr. Yojana Gokhale, Dr. Amol Pawar, Dr. Chaya Shinde*, Dr. Eesha Gokhale*, Dr. Anand Kumtha*, Dr. Lalana Kalekar, Dr. Ravi Shinde, Dr. Divya Lala ; Rheumatology Services, Dept. Of Medicine, and Dept. Of Ophthalmology, Lokmanya Tilak Medical College, Sion, Mumbai, and Dr. Gokhale Clinic and Arthritis Center, Goregaon-W. Mumbai, *Ophthalmologists

Background : Uveitis, a rare, but vision threatening condition is often associated with systemic diseases. All uveitis (other than first attack of acute anterior uveitis and those with infective etiology) are to be treated with immunosuppressants. Rheumatologist can play important role in management of uveitis by identifying associated systemic condition and monitoring immunosuppressants. This study aimed at evaluating outcome of joint venture by rheumatologist, ophthalmologist in treating non-infectious uveitis with immunosuppressants.

Methods : Patients of non-infectious uveitis, seen over 10 years (2006- Feb 2016 ) in a private rheumatology clinic were retrospectively analysed and those from a tertiary centre over 30 months (June 2013-Dec 15) were prospectively enrolled. All patients were screened (clinically and investigations) for systemic disease. Their Vn, IOP, slit lamp, funduscopy findings, complications if any were recorded at first visit, and at 1, 3, 6 months. All, except first attack of AAU were stared on oral Prednisolone 1mg/kg, tapered over next 3-4 months to 5mg and Inj. Methotrexate 25mg/week(switched to oral when inflammation was controlled or by 3 mn), in addition to topicals. Cyclosporine was used as 1st line immunosuppressant in Behcet's disease. TNF-I were used when indicated. Primary outcome was improvement in Vn by two lines, secondary outcome was remission while on oral Prednisolone <5mg/d and Oral Methotrexate <15mg/week.

Results: There were 92 patients of uveitis (139 eyes), 58 retrospective, 34 prospective, M:F:46 :46, average age 38.8 years. Anterior 45.65%, posterior 29.34%, intermediate 18.47, pan uveitis 6.52%. Their mean BCVA (best corrected visual acuity) at presentation was 0.66 (between 6/24 to 6/36 ). Systemic association was found in 41 % patients. Associated diseases were 19.6%.SpA, 7.6% BD, 5.4% sarcoid, 3.3% VKH and JCA 3.3%. Vision improved to 6/6 on Snellen Chart in 54.87%. Two line improvement occurred in 71.7%. Complications like cataract 25%, macular oedema 11%, retinal detachment 7.3%, glaucoma 3.6%. No side effects of treatment were noticed.

Conclusion: Two line improvement occurred in 71.7 % patient with oral steroids and Inj. Methotrexate 25mg/week at 6 months.


  P63 Category: Basic Immunology Top


Telmisartan attenuated inflammation, oxidative stress and RANKL, an osteoclast-related cytokine expression in rat model of adjuvant induced arthritis

Namrata Vadak, Anuradha Majumdar; Bombay College of Pharmacy, Kalina, Mumbai 40009

Background/Purpose: The present study demonstrated the effect of Telmisartan, a selective Angiotensin II Type I receptor blocker (AT1R) in rat model of Rheumatoid Arthritis (RA). Receptor activator of nuclear factor kappa B ligand (RANKL) is a member of Tumour necrosis factor alpha (TNF-α) family, also known as osteoclast differentiation factor. RANKL mediates osteoclastogenesis by binding to receptor activator of nuclear factor kappa B (RANK) present on pre-mature osteoclasts leading to their maturation and survival which aids in bone resorption.

Method: Arthritis was induced in male Wistar rats by immunizing with 0.1ml of Complete Freund's Adjuvant (10 mg/ml) intradermally at the base of the tail on day 0. Treatments with Methotrexate (MTX) (1mg/kg, i.p.) twice a week and Telmisartan (5, 10, 20 mg/kg, p.o.) daily were initiated after the onset of inflammatory signs i.e. from day 12 onwards till day 28. Animals were sacrificed on day 28 and serum and ankle joints were isolated. Clinical parameters evaluated included arthritic scoring, hind paw swelling, body weight changes from day 0 till day 28 as well as radiography and histopathology of ankle joints on day 28. Ankle joint homogenates were analyzed for biochemical parameters such as malondialdehyde, nitrite, reduced glutathione (GSH) levels and activities of catalase (CAT) and superoxide dismutase (SOD). The expression of receptor activator of nuclear factor kappa B ligand (RANKL) in ankle joints was analyzed by reverse transcription polymerase chain reaction (RT-PCR). Tumour necrosis factor (TNF-α), a pro-inflammatory cytokine was evaluated in serum sample by ELISA.

Results: Disease control rats exhibited prominent hind paw swelling, oxidative stress as well as increased RANKL expression and TNF-α level in the ankle joints. Low dose Telmisartan (5 mg/kg) group did not exhibit any significant protective effects in comparison to disease control rats. MTX (1mg/kg, i.p.) significantly reduced hind paw swelling, arthritic scores, levels of malondialdehyde, reduced glutathione, nitrite and TNF-α. Radiographical and histopathological analyses revealed an improvement in joint architecture when compared with disease control rats. Treatment with Telmisartan (10, 20 mg/kg) significantly reduced the arthritic severity indicated by reduced arthritic score and swelling. They also prevented the rise in the levels of malondialdehyde, nitrite, TNF- α. Improvement in antioxidant status was evident by enhancement in catalase and superoxide dismutase activities. Radiographical and histopathological analysis showed restoration of normal joint architecture with Telmisartan when compared with disease control rats. Treatment with Telmisartan down regulated RANKL expression in ankle joint tissue in comparison with disease control rats.

Conclusion:
The study demonstrated that the anti-arthritic effect of Telmisartan in AIA model is associated with inhibition of inflammation and RANKL expression.


  P64 Category: Systemic Lupus Erythematosus Top


Association of TNFSF4 polymorphism with susceptibility to Systemic Lupus Erythematosus in North Indian Population

Vikas Gupta, Avadhesh Pratap, Sandeep Kumar, Rajeev Singh1 , Amita Aggarwal, Ramnath Misra; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow and1 Department of Biochemistry, King George Medical University, Lucknow

Background/Purpose : TNFSF4 gene (located at chromosome 1q25) encodes for TNFSF4 (also known as OX40L protein), a cytokine involved in T-cell and antigen presenting cell (APC) interactions. Increased levels of TNFSF4 lead to increased activation of T and B cells, hence, predisposing to Systemic Lupus Erythematosus (SLE). Single-nucleotide polymorphisms (SNPs) in TNFSF4 gene have been shown to be associated with SLE in European and Chinese populations. We aimed to verify the association of TNFSF4 polymorphism (rs2205960) with SLE in North Indian population and to study the genetic relations between this SNP and sub-phenotypes of SLE.

Methods : 306 SLE patients and 480 controls were enrolled. All samples were genotyped for SNP rs2205960 using TaqMan genotyping assay in Roche LC480 real-time polymerase chain reaction (PCR) system. A case-control association study and a genotype phenotype correlation were performed.

Results : The mean age of the SLE patients was 30.7 years and 94.4% were females. Allelic and genotypic frequencies did not deviate from Hardy-Weinberg equilibrium in the controls (p > 0.05). Allele contrast showed that the variant allele T was associated with increased risk for SLE when compared with the G allele (OR = 1.28, CI = 1.02 - 1.62, p = 0.039). A significant difference was also detected under a dominant model with regard to the distribution of genotype frequencies between SLE patients and healthy controls [(TT + GT) vs GG: OR = 1.37, 95% CI = 1.03 - 1.82, p = 0.034] while no association was observed between SNP rs2205960 and any of the clinical manifestations of SLE.

Conclusion : A single nucleotide polymorphism rs2205960 in TNFSF4 gene predisposes to SLE in North Indian population. Our results are in accordance with the previous studies conducted in European and Chinese populations, indicating that the TNFSF4 gene might be a common genetic factor associated with SLE in multiple ethnic groups.


  P65 Category: Systemic Lupus Erythematosus Top


Utility of urinary Monocyte chemoattractant protein-1 (MCP-1) and ceruloplasmin in diagnosing active lupus nephritis

Kumar C Santosh1 , Kumar Uma2, Suman Shiv Kumar2 , Dinda Amit Kumar3 , Sreenivas Vishnubhatla4 , Mahajan Sandeep5 , Ranjan Piyush1 , Wig Naveet1 ;1 Department of Internal Medicine,2 Rheumatology,3 Pathology,4 Biostatistics, and 5Nephrology, All India Institute of Medical Sciences, New Delhi, India

Background: Nephritis is a common and serious manifestation of systemic lupus erythematosus (SLE) characterized by relapses in 30-50% patients. Performing repeated renal biopsies to assess treatment response or renal flare is not a viable option. This necessitates the need of a biomarker that can detect the activity non-invasively. This study was undertaken to study the utility of urinary biomarker MCP-1 and ceruloplasmin in diagnosing active lupus nephritis and it's role in predicting the treatment response at 6 months.

Materials and Methods: We studied 40 subjects (30 SLE patients and 10 healthy subjects) stratified into 4 groups (10 in each group) i.e active lupus nephritis, lupus nephritis in remission, non-renal lupus and normal healthy controls. Lupus activity was assessed by SLEDAI -2K. Urinary biomarkers (ceruloplasmin and MCP-1) were analysed at baseline and after 6 months of treatment in active lupus nephritis group, whereas in other groups biomarkers were analysed at baseline only.

Results: Urinary levels of MCP-1 at baseline were significantly elevated in active lupus nephritis compared to lupus nephritis in remission, non-renal lupus and normal controls (p=0.001). However urinary levels of ceruloplasmin, even though were found higher in patients with active lupus nephritis but when compared with lupus nephritis in remission, they did not reach to the level of statistical significance(p=0.1). Out of 10 patients of active lupus nephritis who were followed until 6 months, 60% (n=6) showed renal response. Both biomarkers at baseline failed to predict response at the end of 6 months. Both the biomarkers did not perform better than conventional serum markers of disease activity or urine proteinuria on receiver operating characteristic (ROC) analysis.

Conclusion: Urinary MCP-1 can be used to differentiate active lupus nephritis from lupus nephritis in remission but it can not predict treatment response.


  P66 Category: Systemic Lupus Erythematosus Top


Phenotypic and functional characterization of human blood monocytes in SLE

Bidyut Das, Ratnadeep Mukherjee* Rina Tripathy#, B Ravindran* ; Unit of Clinical Immunology & Rheumatology, SCB Medical College Cuttack, *Division of Infectious Disease, ILS, Bhubaneswar, # Department of Biochemistry, SCB Medical College, Cuttack.

Background: Given the importance of monocytes in the pathogenesis of infectious and other inflammatory diseases, functional and phenotypic characterization of monocyte subsets has emerged as a critical requirement, especially in the context of diseases. In the present study, we used a combination of multicolour flow cytometry and multivariate statistical modelling to probe phenotypic and functional aspects of human blood monocytes in healthy individuals and patients suffering from systemic lupus erythematous (SLE).

Methods: We employed multiparameter flow cytometry to reliably select monocytes in human peripheral blood. A high dimensional clustering algorithm was used to automate flow cytometry gating procedures in order to confirm a manual gating strategy. Intracellular cytokine measurements and uptake of GFP - expressing E.coli was scored by flow cytometry. Finally, relative percentages of different monocyte subsets were quantified in SLE patients and the levels were compared with that of normal individuals.

Results: Twenty (20) SLE female patients in the age range of 15 to 45 were included in the study for analyses. Our findings suggest that ficoll purification of blood monocytes leads to a decrease in number of CD14+/CD16- classical monocytes with a concomitant expansion of CD14dim/CD16+ nonclassical monocytes. Moreover, gradient separation also contributed to increased surface expression of CD16. We demonstrate that nonclassical monocytes produce significantly higher levels of proinflammatory cytokines whereas classical monocytes display higher phagocytic activity. Consistent with this, we also report increased percentage of nonclassical monocytes in SLE patients with a concomitant decrease of classical monocytes.

Conclusions: We conclude that 'Classical' monocytes are phagocytic with no inflammatory attributes, 'Non-classical' subtype display 'inflammatory' characteristics on activation and their percentage increases in SLE.




  P67 Category: Systemic Lupus Erythematosus Top


Predictors of development of cardiac tamponade among in-hospital patients with systemic lupus erythematosus: a prospective observational study

Rudra Prosad Goswami1 , Anupam Maity2 , Parasar Ghosh3 , Alakendu Ghosh3; Institute of Post Graduate Medical Education and Research, Kolkata, India

Objective : The aim of the present study was to investigate the prevalence of tamponade among patients with pericarditis related to SLE and determination of its predictors of development.

Methods : In this prospective observational study, we collected clinical, laboratory and therapy related data from all the admitted patients of SLE within August 2014 to March 2016. Pericarditis was diagnosed according to 1999 revised American College of Rheumatology (ACR) classification criteria for SLE. Cardiac tamponade was diagnosed through echocardiography. Following were excluded: requirement of IV or oral antibiotics, anti-tubercular drugs, conditions where non-lupus causes of pericarditis could not be excluded. After univariate analysis variables with p<0.2 were entered into a binary multivariate stepwise logistic regression (MSLR) model for predictors of development of tamponade.

Results : After exclusion of 32 patients, data of 230 patients with SLE (female: 93%, 214/230; mean age 28±9 years, mean disease duration 19±23 months) were collected. Pericarditis was diagnosed in 22.6% (52/230) and tamponade in 5.2% (12/230). Tamponade was the presenting feature in 66.7% (8/12) of patients. Four developed this complication after a mean duration of 30±28 months (12-72 months) following first symptoms related to SLE. All of the patients required emergency pericardiocentesis. The following immunosuppressive drugs were used: pulse methylprednisolone: 4 patients, 1-mg/kg/day prednisolone: 7 patients, 0.5-mg/kg/day prednisolone: 1 patient; cyclophosphamide: 10 patients and azathioprine: 2 patients. None of the patients died. Pleuritis was significantly more common in patients with tamponade (p=0.02). In MSLR only two variables were significantly associated with tamponade: pleuritis (Odd's ratio (OR): 8.98, 95% confidence interval (CI): 1.76-45.71, p=0.008) and hypoalbuminaemia (OR=0.15, 95% CI: 0.02-0.89, p=0.037).

Conclusion : Tamponade occurred in 23% of admitted patients with pericarditis. Development of tamponade was positively associated with concomitant pleuritis and negatively with hypoalbuminaemia.


  P68 Category: Systemic Lupus Erythematosus Top


The (F)utility of Testing for Anti Thrombopoeitin Receptor Antibody profiling in Lupus related thrombocytopenia

Arvind G1 ,,Jayakanthan K1 , Hindhumathi Mohan1 Mahasampath Gowri2 , John Mathew1 , Debashish Danda1 ;1 Department of Rheumatology &2 Department of Biostatistics Christian Medical College, Vellore

Background: Thrombocytopenia occurs in 7-30 % SLE patients. Anti Thrombopoetin receptor antibody (Anti TPO-R Ab) has been reported to be present in 12% lupus related thrombocytopenia is associated with decreased platelet production, megakaryocyte hypoplasia and poorer treatment response. Objective- To study the relation of Anti TPO- R Ab in lupus related thrombocytopenia with respect to bone marrow megakaryocyte density and treatment response at 3 months (defined by manual platelet count >1,00,000/mm3)

Methods: 45 SLE patients satisfying the SLICC/ACR criteria with manual platelet count<1,00,000/mm3 not attributable to any other cause were enrolled in the study. Their demographics, clinical and laboratory parameters along with treatment details were noted. Sera from these SLE patients were tested for Anti TPO -R Ab (Qualitative ELISA,CUSABIO China)

Results: Median manual platelet count was 30,000/mm3 (10,000-90,000 mm3) and Anti TPO- R Ab was positive in 8/45 (17.77%) patients. Bone Marrow Megakaryocyte density was normal/increased in 27/45 (53.33%)patients, decreased in 2/45 (4.44%) patients and not available in 16/45(35.56%) patients. Anti TPO- R Ab was seen in 1/29(3.45%) patients with bone marrow hypoplasia compared to 7/29(24.14%) patients with normal bone marrow (p-0.101). Anti TPO-R Ab was positive in 2/8 (25%) patients without treatment response compared to 6/35 (17.14%) patients with treatment response (p-0.606). Median manual platelet count was 56,250/mm3 (30,000-75,000) vs 30,000/mm3 (10,000-90,000) in Anti TPO-R Ab positive and negative patients respectively (p-0.02). Anti TPO-R Ab positivity was not significantly associated with life threatening bleeding events or lupus related serological parameters.

Conclusion: Our study, unlike previous studies, shows that Anti TPO-R Ab in Lupus related thrombocytopenia patients did not predict a lower platelet count, bone marrow megakaryocyte hypoplasia or poor treatment response


  P69 Category: Systemic Lupus Erythematosus Top


Large Single Centre Experience of Myelitis in Lupus

Sravan Kumar Appani1 , Sunitha Kayidhi1 , Rajendra Vara Prasad Irlapati2 , Liza Rajasekhar3 ; Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background : Myelitis in SLE is rare. We analysed the associations of myelitis in our lupus cohort.

Methods : Medical charts of patients in our institute from 2007 to 2016 were reviewed for a diagnosis of SLE and myelitis. Clinical, laboratory, treatment, outcome details were noted. Complete recovery was defined by absence of motor, sensory deficits and sphincter disturbances 6 months from myelitis onset. Descriptive statistics was used for continuous data. Age, ESR, SLEDAI, interval between disease onset and myelitis was compared between complete recovery vs non recovery group by Mann-Whitney test. Categorical data was expressed by percentage and frequency. Fishers Exact Test was done for association of variables(simultaneous diagnosis of myelitis & lupus, gender, organ involvement, dsDNA, complements, aCL antibodies, CSF and MRI findings, treatment details) with complete recovery. p value <0.05 was considered significant.

Results : Seventeen of 1670 SLE patients had myelitis. Two had infectious and 15 had lupus related myelitis. Mean (SD) age was 24.4(7.6) years and SLEDAI 16.06(7.73). Median (range) disease duration before myelitis onset was 8(190) months. Myelitis occurred at diagnosis of lupus in 7. Four patients died. Clinical manifestations included other NPSLE manifestation in (11/15), nephritis and myopathy(4/15), mucocutaneous (10/15), myocarditis, PAH, pancreatitis (2/15), arthritis (5/15), leucopenia (3/15), thrombocytopenia (6/15), low complements (11/12), dsDNA positive (11/14), aCL antibody positivity in (7/13) patients. CSF revealed elevated protein in 7/8, low glucose and pleocytosis in 4/8 patients. Spinal cord edema in MRI documented in 3/7 patients. Therapy included iv high dose steroids in 14, cyclophosphamide in 11, IVIg in 1, plasmapheresis in 1 patient. Complete recovery was documented in 4, partial recovery in 5 and worsening in 5.

Patients with myelitis at presentation of lupus had significantly more complete recovery (p˂0.05). No other variable was found to be associated with myelitis recovery.

Conclusion : Incidence of myelitis in our cohort was 1.02% with poor prognosis and high mortality. Myelitis occurring early in lupus is associated with better prognosis.


  P70 Category: Systemic Lupus Erythematosus Top


Nailfold microvascular abnormality in systemic lupus erythematosus (SLE) with anti- U1 RNP positivity :A Descriptive Pilot Study

Pramod Prahlad Chebbi1 , Ruchika Goel1 , Ramya J1 , Santhosh Mandal1 , Debashish Danda1 ; Christian Medical College, Vellore

Background: To study nailfold microvascular abnormality in systemic lupus erythematosus (SLE) with anti- U1 RNP positivity (RNP+).

Methods:
Nailfold videocapillaroscopic(NFVC) examination (Optilia mediscope, 200X) was performed in consecutive patients satisfying classification criteria of SLE and mixed connective tissue disease (MCTD), were recruited. Undifferentiated connective tissue disease(UCTD) patients and primary antiphospholipid antibody(APS) patients without RNP positivity were also recruited as disease controls.

Results: Among 23 patients studied (age 38.8±9.08; 22 females), 6, 5, 6 and 6 had SLE with RNP+, SLE without RNP positivity, MCTD and UCTD/primary APS respectively. Total 12 subjects were RNP positive. None of RNP+ patients had normal capillary density. Ramification and giant capillaries were observed exclusively in RNP+ patients [MCTD 83.3% and 100%); RNP+ SLE (66.6% and 33.3%) respectively] and enlarged capillaries were more frequent in RNP+ (100%) than RNP negative patients (27.7%). Haemorrhages were observed in 91.6% of RNP+ patients as compared to 54.5% in RNP negative patients. Four out of six SLE patients with RNP+ did not have raynauds clinically but all had abnormal microvascular changes comparable to MCTD patients but with lesser severity.



Conclusion: Anti-U1RNP in lupus is associated with significant microvascular changes even in the absence of Raynuads. Intensity of changes is more profound in MCTD. This is the first data of NFVC on Indian patients.


  P71 Category: Systemic Lupus Erythematosus Top


A study of clinical & pathological parameters and treatment outcomes in subjects of lupus nephritis with crescent

Manish Rathi, Gadella Varun Teja, Aman Sharma1 , Ritmabhra Nada, Gupta KL; Department of Nephrology,1 Rheumatology & Histopathology, Post Graduate Institute of Medical Education & Research, Chandigarh, India

Title: A study of clinical & pathological parameters and treatment outcomes in subjects of lupus nephritis with crescent

Background:
Crescentic lupus nephritis (CLN) is the most severe form of renal involvement in systemic lupus erythematosus (SLE). We aim to study the significance of crescents on the presentation and treatment outcomes and the factors associated with outcomes.

Materials and Methods: Renal biopsy confirmed lupus nephritis cases were included and divided into four subgroups: Control Group (no crescents), Group 1 (<25% crescents), Group 2 (25%-50% crescents), and Group 3 (≥50% crescents). Clinical details were noted, and the renal biopsy was reviewed for type of crescents, underlying glomerular changes, activity and chronicity indices, tubulointerstitial and vascular damage. Outcomes were assessed at the end of six months.

Results: A total of 150 patients (136 females, 14 males) with mean age of 31.2 ± 10.23 years were included. Of these, 85 (56.7%) were in control group, 31(20%) in group 1, 13 (8.7%) in group 2 and 21 (14%) in group 3. Group 3 patients had more rapidly progressive renal failure, high serum creatinine (p = 0.001), high activity and chronicity indices (p <0.005), without any significant difference in proteinuria or disease activity. Treatment failure and death were more and complete remission was less in group 3 at follow-up. On linear regression, cellular and fibrocellular crescents were independent predictors of initial and follow-up serum creatinine (p <0.005), while fibrous and fibrocellular crescents were independent predictors of follow-up proteinuria.

Conclusion: CLN was associated with more severe renal presentation and poor treatment outcome. The severity of presentation and adverse treatment outcome increases with increase in percentage of crescents.


  P72 Category: Systemic Lupus Erythematosus Top


Diagnostic Utility and Clinical correlation of Anti dsDNA, Anti Nucleosome antibodies and Complement levels in Systemic Lupus erythematous - A prospective study

Sabarinath S1 , Rama Bhat2 , Indira Bairy3 ; General medicine & Microbiology, Kasturba medical college, Manipal University, Manipal

Background: It is proven that nucleosomes are the major immunogen in the pathogenesis of SLE. The present study focusses on the analysis of clinical activity of SLE using SLEDAI 2K30 score, diagnostic utility of anti dsDNA, anti Nucleosome antibodies and complement levels, and its correlation with SLEDAI 2K30 Score.

Methods: 73 newly diagnosed SLE cases, fulfilling the ACR criteria was included. Excluded were those on immunosuppression, those with active infection, pregnancy, liver disease, overlap syndrome. Using SLEDAI2K:30 score,cases were classified as inactive (Score < 5) and active disease (Score > or equal to 5). ANA testing by immunofluorescence, ELISA of anti dsDNA and antinucleosome antibody, complement assay by nephelometry were done. SLEDAI2K30 score was correlated with antibody titres and complement levels.ROC curve plotted to define the cutoff for anti dsDNA, antinucleosome and complement levels in active SLE.

Results: Among the 73, 54 had active and 19 had inactive disease. ANA IF was positive in all cases, most common pattern being Homogenous. For diagnosing SLE, Sensitivity of anti dsDNA and anti Nucleosome antibody was found to be 90.4% and 76.7% respectively [ref fig 1]. Anti dsDNA had a stronger positive correlation with SLEDAI2K30 (r=0.725; p=0.01) than antinucleosome antibody (r=0.618 p=0.01).From the ROC curve for antibodies in active SLE cases [ref fig 2], Sensitivity and specificity of anti dsDNA was 83.3% and 95.7% for the cut-off of > 480IU/ml; 87% and 95.7% for antinucleosome for the cutoff of >79IU/ml. C3 (r=-0.601 p=0.01) had a stronger negative correlation with SLEDAI2K30 than C4 (r = -0.523 p= 0.01). Sensitivity and specificity of C3 was 81.5% and 90.5% for the cut-off of < 52mg/dl ; 74% and 90.5% for C4 for the cutoff of <9mg/dl in active SLE cases.

Conclusion: Anti Nucleosome antibody could be an additional and better tool for diagnosing active SLE cases.

ATTACHMENT 1:

Figure1 illustrating anti dsDNA titres and antinucleosome titres and disease activity in SLE patients, [active disease SLEDAI2K30 > or equal to 5 ; inactive disease with a score <5]



ATTACHMENT 2

Figure 2 Illustrating Receiver operator Characteristic curve in active SLE cases for anti dsDNA and anti nucleosome antibodies


  P73 Category: Systemic Lupus Erythematosus Top


"Clinical profile and long term outcome of neuropsychiatric lupus from a tertiary care center in South India"

Bhuvanesh Mahendran, Rajeswari Sankaralingam, Balaji Chilukuri, Saranya Chinnadurai, Saravanan M; Institute of Rheumatology, Madras Medical College, Chennai, India

Background: Neuropsychiatric manifestations represent severe involvement in SLE. The long term outcome of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) is not clearly defined.

Objective:
To investigate the neuropsychiatric (NP) manifestations in SLE and identify predictive factors for clinical outcome.

Methods:
Retrospective review of SLE patients between 1996 and 2016 was done. NP manifestations, SLICC/ACR damage index, SLEDAI, MRI findings, immunological investigations and mortality rates were included for analysis. Individual NP manifestations were defined using the proposed ACR 1999 nomenclature and case definitions for neuropsychiatric lupus syndromes. Clinical outcome of NPSLE was classified based on the Neuwelt's classification.

Results:
Of the 490 patients with SLE, 154 patients had at least one defined NP manifestation. The most common NP manifestation was seizure (38.2%) followed by mood disorder (32.6%). 85 patients (55%) had more than one NP manifestation. SLICC/ACR damage index was significantly higher in patients with NPSLE compared to SLE without NP manifestations. 74 patients (48%) had more than one NP manifestation and in these patients had a higher SLICC/ACR damage index when compared to patients with only one NP manifestation. Most common MRI findings were multiple T2 hyperintense lesions (26%) followed by infarcts (24%). Cyclophosphamide was the most common immunosuppression used followed by mycophenolate mofetil. Multivariate analysis showed that high SLICC/ACR damage index and more than 2 sets of NP manifestations at presentation were the strongest predictors of poor outcome.There were total of 36 deaths recorded in the group with NPSLE.

Conclusion
: Higher SLICC/ACR damage index and more than two sets of NP manifestations at presentation were associated with poor outcomes in NPSLE.


  P74 Category: Systemic Lupus Erythematosus Top


Correlation Between Antidsdna, Complement Component C3, C4, And Disease Activity In Systemic Lupus Erythematosus In A Tertiary Care Center.

Therese Mary Dhason, Euphrasia Latha Jairaj, Rajeshwari Sankaralingam, Saranya C, Kumudha Manoharan, Radhabhai C; Rajiv Gandhi Government general hospital,Madras Medical college,Chennai

Background: In many cases of SLE there is a relationship between disease activity and the serum titre of antidsDNA. Acute exacerbations of the disease can be associated with low serum complement levels.

Objective:
To find out the correlation between antidsDNA, C3, C4 and disease activity in systemic lupus erythematosus.

Materials and Methods:


Duration of study: January 2016 to June 2016.

Type of study: Cross sectional study.

Inclusion criteria: Patients with active disease.

Study group:

Total n 300

Patients with disease activity n 100.

Patients with inactive disease n 100.

Healthy controls n 100.

Method:

ELISA for antidsDNA assay.

Radial Immunodiffusion for C3, C4 assay.

Results: High titre antidsDNA was present in 48 out of 100 patients with active disease. Mean SLEDAI of patients with high titre antidsDNA was 23.0833 ± 3.80276 which was statistically significant (p < 0.001), Mean SLEDAI of patients with very low C3 was 21.4314 ± 4.88776 and mean SLEDAI of patients with very low C4 was 21.1250 ± 4.82882 which were statistically significant (p < 0.001). In the study group high antidsDNA and low C3, C4 levels has disease markers is statistically significant (p<0.001) in patients with active disease.

Conclusion: AntidsDNA and C3, C4 assay can be adopted as disease activity markers on patients with active Lupus. ELISA for antidsDNA can be done, as the procedure is simple and cost effective. Radial immunodiffusion for C3, C4 assay can be done where Nephelometer is not available as the procedure is simple.

Tables: COMPARISON OF antidsDNA WITH SLEDAI




  P75 Category: Systemic Lupus Erythematosus Top


Improved Outcomes in High Risk Lupus Pregnancies: Usefulness of a Protocol Based Multidisciplinary Approach in Kerala, India

V Ravindran1, 4 Bhadran S2 , Reshma V3 ;1 Departement of Rheumatology &2 Department of Obstetrics, National Hospital, Calicut,3 Department of Obstetrics, Indira Gandhi Hospital, Thalassery,4 Centre for Rheumatology, Calicut

Background/Purpose: In patients with SLE who had previous adverse obstetric outcome(s) the risk of an adverse outcome in subsequent pregnancy rises. The primary objective of this study was to assess the impact of a protocol based multidisciplinary care on pregnancy outcome(s) in a prospective cohort of patients with SLE who had previous adverse obstetric outcome(s).

Methods: Between March 2010 and March 2016 all patients with SLE with previous at least one adverse obstetric (either maternal or foetal) outcome desirous of having more children were prospectively enrolled. All patients had in-depth preconception counselling and were prospectively followed throughout the pregnancy as per the protocol with multidisciplinary team input. Briefly the protocol comprised of pre natal counselling, pre & post natal drug and disease status review, risk stratification, periodic ante-natal visits for the monitoring of pregnancy (including with obstetric ultrasounds) and disease, 2D echocardiogram at weeks 18 and 32 if Ro/La were positive and post natal contraception advice. Therapeutic changes were made as necessary at each visit.

Results: Fifty-three patients (age mean±SD years; 28±3) were enrolled. Previous poor obstetric outcomes were: miscarriage(s) in 30, MTP in 9, preterm labour with IUGR in 9, intrauterine death, still birth in 2 each and pre ecalapmsia in 1. Nine patients had secondary APS and 14 had both or either Ro/La positive. Nine had lupus nephritis (LN); > 6months ago in all. There were 44 (85%) live births (10 LBW; instrumentation or caesarean section in 28). Eight patients had miscarriages. One decided against becoming pregnant after the initial counselling. Sixteen patients (30%) had lupus flare (9 mild, 5 moderate and 2 severe based on SLEPDAI).

Conclusion: Majority of patients in our prospective cohort had acceptable pregnancy outcome. This highlights that in India we need to provide integrated, innovative solution to the complex need of SLE pregnancies.


  P76 Category: Systemic Lupus Erythematosus Top


Effect of gender on clinical presentation and serological profile (ENA, APLA) in SLE patients : Prospective study

Muzaffar Bindroo, Dhiren Raval, Gayatri Ekbote, Natasha Negalur, Naval Mendiratta, Shruti bajad, Vinay Singal, Rajiva Gupta; Medanta- the Medicity, Gurgaon, Haryana 122001

Background: Although systemic lupus erythematosus (SLE) affects mostly females(9:1). many studies have shown that differences were observed between male and female lupus patients.Few studies have revealed that men with lupus display decreased musculoskeletal symptoms, photosensitivity, oral ulcers and RP and increased renal, hematological, thrombosis and serological manifestations and others did not found any difference. We sought to explore whether gender exerts an influence on the clinical presentation and serological profile (ENA & APLA ) in our cohort.

Methods:
After taking proper informed consent and IRB ethical clearance, all SLE patients(Inpatients and outpatients) diagnosed as per ACR 1997 criteria visited between August 2015 to August 2016 period,were included. After taking history and examination, ENA profile (using Belgium based BlueDiver Quantrix kit - Immunodot semiquantitative ELISA method) and APLA profile(LAC, ACL-IgM, ACL-IgG, B2GpI-IgM, B2GPI-IgG) was done. Clinical presentations and serological profile was compared between two groups and P value was calculated.

Result:
Our cohort consisted of 24 men (14.90 %) and 137 women (85.09 %). Mean age of males were 40 yrs (Range 18-71) and females 36 yrs (19-69). For manifestations at enrollment, males presented more frequently with thrombocytopenia (p = 0.00), nephritis (p = 0.04), azotemia (p=0.02),myocarditis (p = 0.030) and NBTE (p0 = 0.001) and females presented more frequently with Photosensitivity (p=0.00), malar rash (p= 0.035), excessive hair fall (p= 0.00) and uveitis (p=0.04). However, the constitutional, serositis, CNS, vascular, gastrointestinal, complement, ENA and APLA profile data (p= > 0.05) showed no significant differences between the two groups at enrollment.

Conclusion:
Our study suggests that there are major clinical differences between male and female SLE patients without any serological differences. Future SLE studies will need to consider large cohort to understand these differences.


  P77 Category: Systemic Lupus Erythematosus Top


Cardiac involvement in SLE and its association with autoantibodies (ENA-profile and APLA profile) Prospective study

Dhiren Raval, Muzaffar Bindroo, Gayatri Ekbote, Natasha Negalur, Naval Mendiratta, Shruti bajad, Vinay Singal, Rajiva Gupta; Medanta- the Medicity, Gurgaon, Haryana 122001

Background: Cardiac involvement is very common in SLE, and carries high morbidity and mortality. There is association of autoantibodies like anti-Ro, anti-La and APLA to cardiac involvement.We plan to study correlation of ENA profile and APLA with cardiac involvement in Indian lupus patients.

Method: All the patients who attended our OPD between August 2015 to August 2016 and fulfilled ACR criteria for SLE were included. ENA and APLA profile was done. Those who had clinical/Lab.findings suggestive of cardiac involvement were evaluated by 2DEcho. This study done after approval and ethical clearance from IRB.

Result: Total number of SLE patients 161.Female 137 (85.09%) and Male 24 (14.90%). 90 patients subjected to 2DEcho out of which 45 (50%) had cardiac involvement.



Isolated Pericarditis seen in 14 (31.11%) and myocarditis 12 (26.66%), Myocarditis + NBTE 6(13.33%), Myocarditis + pericarditis 1(2.22) Valvular dysfunction 5(11.11).

Conclusion: We found LAC, Acl IgM and Acl IgG were more commonly detected in SLE patients with cardiac involvement than in SLE without cardiac involvement. However was not statistically significant. Few western studies have shown association of Ro and La with cardiac manifestation in SLE, we did not find significant association. Our study revealed myocardial dysfunction was most common cardiac manifestation followed by pericarditis.


  P78 Category: Systemic Lupus Erythematosus Top


Clinicopathological Correlation with Outcome in Patients with Lupus Nephritis - A Retrospective Cohort Study

Jha Saket, Kumar Uma1 , Dinda AK2 , Soneja Manish, Ranjan Piyush, Ranjan Rajiv, Singh Geetika3 , Sreeniavs V4 , Mahajan Sandeep5 ; Departments of Medicine,1, 2 Rheumatology, 3Pathology,4 Biostatistics and5 Nephrology, All India Institute of Medical Sciences, New Delhi, India

Background/Purpose: The purpose of this study was to evaluate the clinical, laboratory and histological predictors of chronic kidney disease (CKD) in patients with lupus nephritis (LN) on long term follow up at a single centre in India

Methods:
The retrospective cohort included 50 patients with kidney biopsy done during 1981 to 2008 and who were in follow up during September 2013 to June 2015. We retrospectively analysed the clinical, laboratory, treatment response and histological indices of patients from the records. Glomerular filtration rate was measured at follow up using Tc99 DTPA scan. The parameters which predicted the risk of developing CKD were identified by univariate and multivariate logistic regression analyses.

Results: In this study majority (92%) were female. The female to male ratio was 11.5:1. The mean duration of lupus nephritis in this study was 13.3 ± 5.9 years. Out of 11 (22%) patients, who developed CKD (GFR <60 ml/min/1.73 m2), all were, WHO class III, IV, V lupus nephritis. 3 out of 15 class III (20%), 6 out of 24 class IV (25%) and 2 out of 6 class V (33%) progressed to develop CKD. The probability of developing CKD was trending with delay in onset of LN after SLE, hypertension, pleural and pericardial effusion, raised creatinine (1.2 mg/dl) at 6 and 12 months of LN diagnosis. On multivariate analysis, however, only hypertension and raised creatinine at 12 months with odds ratio of 12 (95% CI 1.04 - 137.67, P = 0.046) and 26.3 (95% CI 1.7 - 406.3, P = 0.019) respectively were seen as an independent predictor of CKD.

Conclusion:
The study concluded that persistent hypertension and persistent rise in creatinine at 12 months of treatment are independent predictor of development of CKD in Indian patients


  P79 Category: Systemic Lupus Erythematosus Top


A retrospective, observational study of low-dose cyclophosphamide vs high-dose cyclophosphamide vs mycophenolate mofetil in the treatment of lupus nephritis

Geeta Bali Sircar1 , Rudra Prosad Goswami1 , Anupam Maity2 , Hiramanik Sit3 , Alakendu Ghosh4 , Parasar Ghosh4 ; Institute of Post Graduate Medical Education and Research, Kolkata, India

Objective: To compare the efficacy in terms of 24-week remission rate and safety of three different induction strategies in patients with lupus nephritis (LN).

Methods: Records of patients with LN, treated between 2014 and 2015, were analysed retrospectively. Three different treatment approaches were compared: 1) high-dose monthly intravenous cyclophosphamide for 6 doses (NIH), 2) low-dose fortnightly intravenous cyclophosphamide for 6 doses followed by daily oral azathioprine (2-mg/kg/day) (EUROLUPUS) and 3) daily oral mycophenolate mofetil (2-3 gm/day) (MMF). Primary outcome measures analysed were achievement complete and partial remission (CR and PR, respectively) by 24-weeks, defined as: complete remission (CR): proteinuria <500-mg/24h and normal renal function; PR: ≥50% reduction in proteinuria to subnephrotic levels and normal renal function. Secondary outcome measures analysed were: systemic lupus erythematosus disease activity index (SLEDAI) and adverse events (AE).

Results: Records of 156 patients with LN (female 95.5%, juvenile 12.2%, mean age 25.9 ± 8.5 years, median duration 6 months (inter-quartile range 15) and mean SLEDAI: 18.4 ± 7.1), treated with NIH in 94/156 (60.3%), EUROLUPUS in 24/156 (15.4%), MMF in 38/156 (24.4%), were analysed. Baseline characteristics were similar except longer disease duration in patients receiving MMF. At 24-weeks CR was achieved in 104/156 (60.7% total, EUROLUPUS: 13/24, 54.2%; MMF: 25/38, 65.8%; NIH: 66/94, 70.2%, p=0.33) and PR in 28/156 (17.9% total, EUROLUPUS: 5/24, 20.8%; MMF: 4/38, 10.5%; NIH: 19/94, 20.2%, p=0.39). At least PR was achieved in 85/94, 90.4% (NIH), 29/38, 76.3% (MMF) and 18/24, 75% (EUROLUPUS) (p=0.04). Patients on MMF had higher SLEDAI (MMF: 4.4 ± 5.1, EUROLUPUS: 4 ± 3, NIH: 2.6 ± 3, p=0.029) at 24-weeks. Patients on MMF had more frequent gastrointestinal AEs (44% vs. 5%) but other AEs were similar.

Conclusions: High-dose intravenous cyclophosphamide still offers the highest chance of remission in patients with LN without any added adverse events.


  P80 Category: Systemic Lupus Erythematosus Top


Varied presentations of Pancreatitis in Systemic Lupus Erythematosis - A Case Series.

Sheba Charles B, Anu Mohan Desai, Vineeta Shobha; St. John's Medical College, Bangalore .

Background/Purpose: Acute pancreatitis associated with SLE is relatively uncommon with an estimated annual incidence of 0.4 - 1.1 per 1000 patients. 2% incidence has been reported by a case series from South India. Patients are typically female and the association between SLE and Pancreatitis is usually within the first 2 years of disease onset.

Results: We report 6 cases of acute pancreatitis associated with SLE with various clinical presentations, from a period of 2014 to 2016. Pain abdomen was the predominant symptom in all the cases. 5 out of 6 patients were females. All the patients had low complement levels. 4 out of the 6 cases had associated pancytopenia where as 5 patients had inflammatory polyartharlgia. 2 patients had associated pleural effusion while 2 patients had neuropsychiatric features. 1 patient presented with AIHA and pancreatitis which has been reported rarely. All the patients were APLA negative.

Conclusion:
The presence of pancreatitis in SLE has been reported to have up to 40% mortality, earlier. However with early diagnosis and treatment including high dose of steroid and supportive care, the prognosis appears to be very good, as noted in our case series. Pancreatitis may sometimes present after initiation of steroid, however the temporal association and the simultaneous presence of other clinical features of SLE help in decision making.


  P81 Category: Systemic Lupus Erythematosus Top


Prospective observational single-center study to evaluate the effectiveness of treating lupus with rituximab and mycophenolate mofetil with minimal oral steroids

Srinivasa.C, Natasha F, Shailee C, Dharmanand BG; Sakra World hospital, Bengaluru, Karnataka

Introduction: Treatment options for systemic lupus erythematosus (SLE) and lupus nephritis (LN) have high associated morbidity and mortality. Side effects, particularly from long-term corticosteroid usage, limit patient adherence, with subsequent impacts on treatment efficacy. SLE is an archetypal antibody-mediated disease and hence promoting B cell depletion appears to target a critical pathophysiological pathway. Trails with rituximab has shown mixed results.

Material and Methods: 12 patients with SLE who were not willing to take cyclophosphamide(CYC) or candidates not suitable for CYC, from Rheumatology clinic, Sakra World Hospital, Bangalore, between Jan 2015 to march 2016, were included in the study. All patients completed 6 months of follow-up. Patients were treated with 2 doses of rituximab (1 g) and methyl prednisolone (500 mg) on days 1 and 15, and maintenance with mycophenolate mofetil (2000mg) and low dose prednisolone (< 7.5 mg) which was tapered off slowly

Results: Among the 12 patients, 10 were females and 2 males. Mean age of the patient is 24.5 years. Mean duration of disease is 24 months. 9 patients received the rituximab for lupus nephritis, 1 for mesenteric vasculitis, one for CNS vasculitis, one for severe cutaneous vasculitis with pancytopenia. Average SLEDI at the time of infusion is 14. Average SLEDI after 6 months is 4. 6 of 9 patient attained renal remission, 2 patients had partial remission and one patient died due to infection and uncontrolled disease (Renal) after 15 days of infusion. 2 vasculitis patients improved completely. One patient required second dose of rituximab in view of recurrence of cytopenia. Two patients had infection requiring hospitalization and one patient had severe bradycardia during the infusion.

Conclusion:
In our prospective single Centre experience suggests that Rituximab is an effective option for treating severe lupus and steroid usage could be significantly reduced and even stopped.


  P82 Category: Systemic Lupus Erythematosus Top


Cross sectional data on early lupus cases; is SLEDAI good enough to pick up major organ involvement in lupus ?

Dr. Nibha Jain1 , Puja Srivastava2 , Sapan Pandya2 ; V.S. General Hospital, Ahmedabad

Background/Purpose: We wanted to study demographic and clinical profile of early lupus patients which we defined as less than one year versus those more than one year. Most studies define lupus flare as SLEDAI > 6. We wanted to look if SLEDAI > 6 included all patients where a bedside diagnosis of major organ or organ threatening lupus was made.

Methods:
This is an observational retrospective study conducted at a Government hospital and medical college over the period October 2015 to august 2016. The two groups early and late (< 1 year and > 1 year) and diagnosis of major organ involvement based on SLEDAI > 6 versus bedside clinical diagnosis of major organ disease (defined as renal, CNS, mononeuritis multiplex, life threatening hematologic or GI or pulmonary invovlement) were compared. We compared all demographic and clinical variables.

Results:
Total 48 patients (46 females and 2 male) with mean age of 28 years + 8.6 were assessed. Mean SLEDAI was 5.6+ 5.4 with 24 having muco-cutaneous invovment, 27 with fever, 33 arthralgia, 4 serositis, 1 gangrene, 1 myocarditis and 11 were biopsy proven nephritis.



Conclusion : Although numbers are small for a fair comparison, we found no major differences in demographic or clinical profile of patients of early (< 1 year) or late lupus except arthralgias and alopecia which were a later manifestation while anemia and myalgia were more in early disease.. Inferring on major organ involvement flare by SLEDAI score might miss early nephritis cases.


  P83 Category: Systemic Lupus Erythematosus Top



Lupus pneumonitis - clinical characteristics and outcome of therapy - A retrospective review from a tertiary care center

Vignesh Mantharam, Rajeswari Sankaralingam, Balameena Selvakumar, Saravanan Mayilsamy, Mythili Seetharaman, Saranya Chinnadurai, Balaji Chilukuri; Institute Of Rheumatology, Madras Medical College

Background: Lupus pneumonitis(LP) is an uncommon severe manifestation of Systemic Lupus Erythematosus (SLE). The aim of this study was to assess the characteristics of LP and its outcome of treatment.

Methods:
We retrospectively reviewed the data collected from patients with SLE. SLE patients presenting with fever, cough and dyspnoea along with compatible radiological features, negative sputum and BAL cultures were diagnosed as LP. The characteristics of patients with LP were compared to SLE patients without LP and outcome to therapy was also recorded.

Results:
Of the data obtained from 117 SLE patients between Jan 2015 and July 2016, 15 patients(12.3%) had lupus pneumonitis. 14 patients were females and the mean age of presentation was 27.33±9.25 years with eight patients(53.33%) having LP at the initial presentation. Four patients had mMRC grade IV dyspnea and two needed mechanical ventilation. Lupus pneumonitis was associated with higher prevalence of palatal ulcer, delirium, polyserositis as compared to those SLE patients without LP (p<0.05), though no difference was observed in cutaneous, musculoskeletal manifestations, myocarditis, nephritis, thrombotic events and other neurological deficits. Patients with LP had significantly higher acute phase reactants, severe anemia, hemolysis, lymphopenia, thrombocytopenia (p<0.05), results summarized in Table 1. Immunological profile revealed high prevalence of hypocomplementemia, high anti-dsDNA titres and positive anti-SSA antibodies. 14 patients responded to treatment with high dose steroids and cyclophosphamide and one expired after 4 weeks of treatment. Of those who responded to initial therapy, two relapsed and six developed residual lung disease.



Conclusion:
Prevalence of lupus pneumonitis is higher in our group. Lupus pneumonitis is associated with higher prevalence of palatal ulcer, polyserositis, ACS, hematological abnormalities, hypocomplementemia, anti-SSA antibodies and lower prevalence of nephritis. Standard therapy with steroids and cyclophosphamide along may reduce mortality.


  P84 Category: Systemic Lupus Erythematosus Top


Late-onset systemic lupus erythematosus: Single centre experience

Muzaffar Bindroo, Dhiren Raval, Gayatri Ekbote, Natasha Negalur, Naval Mendiratta, Shruti Bajad, Vinay Singal, Rajiva Gupta; Medanta- the Medicity, Gurgaon. Haryana. 122001

Background: Systemic lupus erythematosus (SLE) is uncommon after the age of 50 years, and studies of elderly patients with SLE are scarce. We conducted the current study to analyze presentation and serological profile (ENA & APLA ) of patients with late-onset SLE (> 60 yrs) and to compare them with those of younger patients with SLE.

Method
: After taking proper informed consent and IRB ethical clearance, all SLE patients (Inpatients and outpatients) diagnosed as per ACR 1997 criteria visited between August 2015 to August 2016 period,were included. After taking history and examination, ENA - profile (using Belgium based BlueDiver Quantrix kit - immunodot semiquantitative ELISA method ) and APLA profile(LAC, ACL-IgM, ACL-IgG, B2GpI-IgM, B2GPI-IgG) was done. Clinical presentations and serological profile was compared between two groups and P value was calculated.

Result : Our cohort consisted of 7 (4.34 %) ≥ 60 yrs and 154 (95.6%) < 60 yrs.The female to male ratio was smaller in the late-onset SLE group (2.5:1). Clinical features found more frequently in late-onset SLE at enrolment were myositis (p = 0.007), myopathy (p = 0.004), vasculitic rash (p = 0.007), ILD (P= 0.007), mononeuropathy (0.001),mesenteric vasculitis (p= 0.04) and feature found less frequently was uveitis (0.001). However no differences were observed in relation to constitutional, arthritis, renal, hematological and serological (ENA & APLA) profile at enrolment.

Conclusion: Our study suggests that there are major clinical differences between old and young SLE patients without any serological differences. Future SLE studies will need to consider large cohort to understand these differences.


  P85 Category: Systemic Lupus Erythematosus Top


Rituximab in Severe Lupus Nephritis

Kakade Girish, Samant Rohini, Balakrishnan C, Sagdeo Parikshit, Shah Romi, Joshi Manohar; P D Hinduja National hospital & Medical Research Centre, Mumbai.

Introduction: Despite aggressive immunosuppressive treatment, ESRD occurs in 26% of lupus nephritis patients. Rituximab has emerged as an alternative when disease is severe or resistant.

Objective: To describe clinical profile and outcome of patients of severe lupus nephritis who received rituximab.

Methods:
This was a retrospective study of patients who received rituximab for severe/ resistant biopsy proven lupus nephritis. Demographic profile, clinical, laboratory and treatment details were noted.

Renal response criteria were defined as follows :

  1. Improvement: At least 50% reduction in urinary protein: creatinine ratio
  2. Partial response: A + urinary protein: creatinine ratio of 0.5-2.0 & < 25% decline in baseline GFR
  3. Complete response: A + urinary protein: creatinine ratio <0.5 & serum creatinine < 1.4 mg%


Results: Data for 14 patients (1 male & 13 females) were collected through review of their medical record files. Mean age at diagnosis was 25.64 yrs & mean duration of follow up was 101.36 months. Immunosuppressants used prior to rituximab were mycophenolate, cyclophosphamide & azathioprine in 11, 9 & 7 patients each. Mean urinary protein: creatinine ratio prior to and after rituximab was 6.81 and 1.85 respectively. Mean GFR prior to & after rituximab was 62.21 & 76.29 respectively. 10 patients had either significant improvement in proteinuria or partial / complete response after rituximab. All 4 patients with GFR > 75 ml/min, responded well to rituximab therapy. 3 patients developed serious infections within 6 months of rituximab, 1 was tubercular meningitis. 2 patients developed solid organ malignancies on follow up.

Conclusions: Most patients with resistant lupus nephritis had partial response with rituximab and best response was seen at 6 months. All patients who didn't respond, had impaired GFR prior to rituximab therapy. Majority of patients had class IV lupus nephritis as per ISN/RPS criteria.


  P86 Category: Systemic Lupus Erythematosus Top


SLE & Rheumatologist: a patient perspective

Nahar Naisar, Nahar Prachi; Arham Rheumatology Center, Nasik, Maharashtra

Background/Purpose: Inadequate health literacy is an independent risk factor for poor health outcomes, including higher hospital admission rates, medication nonadherence and mortality. Most rheumatologic diseases require lifetime therapy, the consequences of nonadherence can be deleterious. SLE is chronic multisystemic autoimmune disorder. This study assessed knowledge, attitude & practice in SLE and quality of life.

Methods: Subjects of SLE were identified. All patients fulfilled 1997 ACR SLE criteria. It is cross sectional random onetime assessment was done. Predecided questionnaire was administered. Clinical and investigations data was extracted pertaining to initial examination and follow up. We assessed the tender joint count, swollen joint count, health assessment questionnaire (modified CRD Pune version), pain visual analog scale (pain VAS), patient global assessment (on 10mm visual analog scale), Physician general assessment (on 10mm visual analog scale), SLEDAI, CDAI. Standard investigations, immunoblot to assay autoantibodies were done. Informed consent taken.

Results: 42 patients of SLE were identified in the period from June 2015 to August 2016. Out of 42 patients, 30 patients volunteered for this study. Baseline characteristic showed female dominance (96.7%) & average age of onset is 29. Average HAQ at presentation was 6.26, SLEDAI was 12.8 & CDAI was 12.5. Questionnaires' as SLE is autoimmune disorder (84%), SLE is limited skin (26.3%), SLE is curable (10.5%), children get affected (36.8%), problems in conceiving (36.8%), aerobic exercises is useful (63.1%).

Conclusion: Baseline KAP & on follow up KAP may useful tool as therapeutic intervention. We need prospective large scale studies in these area. We can help patients to improve practice and decrease the burden of complications and their related expenditures by increasing their knowledge.


  P87 Category: Systemic Lupus Erythematosus Top


An Audit of Indoor Admission of Lupus Patients In A Government Hospital

Mitesh Makwana1 , Nibha Jain2 , Puja Srivastava3 , Sapan Pandya4 ; V.S. General Hospital, Ahmedabad

Background : Lupus patients continue to get complications either in the form of infections or disease flares despite new treatment options. We wanted to look at the reasons for admission in our patients presenting to the OPD or emergency.

Methods : Ours was a retrospective observational study. We retrieved files of all lupus patients admitted to our wards or ICU from April 2014 to Aug 2016. Demographic and clinical variables were analysed using descriptive statistics and we also looked at reasons for admission, days of admission, re-admissions amongst these patients.

Results: There were 30 admissions during the period, all females. While 27 were admitted to wards, 3 needed ICU admissions. 23 patients were admitted with disease flares for control of disease, 3 newly diagnosed for work up and 3 for infusions. 1 patient was admitted with epistaxis and severe anemia, unrelated to the disease. Maximum number of re-admissions per patient were 5 and minimum re-admission was 1.

Demographic features of admitted patients:



Clinical features of admitted patients: (out of 30 patients)



Conclusion : Most of our patients were admitted with disease flares and none with infections. More data with longer follow up is needed to include all patients with complications some of whom may not be reaching us.


  P88 Category: Systemic Lupus Erythematosus Top


 Salmonella More Details infections in patients with systemic lupus erythematosus: a case series

Srujana A1 , Varaprasad IR2 , Rajasekhar L3 ; Nizam's institute of medical sciences, Hyderabad, India .

Background: Systemic lupus erythematosus (SLE) patients are highly susceptible to infections including salmonella both typhoid and non-typhoid. We aim to present our experience regarding the manifestations and outcomes of Salmonella infections in SLE patients.

Methods: Medical records of SLE patients from 2012-2016 are reviewed. Patients with documented culture positive salmonella infection were included. Descriptive statistics are used to present the data.

Results: Fifteen patients (11 females) with salmonella, from 798 SLE patients are included. Mean age (± SD) was 25.8 (± 9.23) years with average SLE disease duration of 3.7 (± 2.9) years to the time of Salmonella infection. Most common presentation is fever (13/15), dysuria (7/15), arthritis (4/15), abdominal pain (5/15), vomiting (3/15), and diarrhea (3/15). Four had septic arthritis (26%), one had concomitant osteomyelitis. Soft tissue abscess and bone infarcts in two each (13%). Sepsis syndrome in two (13%). Mixed infections with Salmonella were seen in five patients. Fourteen patients (14/15) were on steroids and seven received more than 10 mg/day of prednisolone. Ten of fifteen patients received antecedent immunosuppression S.typhi was isolated in four (all from blood), S.typhimurium in six (two from blood, two from synovial fluid, one from urine, both synovial fluid and urine in one patient), S.enterica in five (two from blood, two from urine and one from both synovial fluid and soft tissue abscess). All had a SLEDAI of >4 with active nephritis in seven (46%), arthritis in four (26%), myositis two (13%), oral ulcers in three (20%), positive dsDNA binding in eleven (73%), lymphopenia in twelve (60%), renal insufficiency in four (26%). Managed with antimicrobials, adjunct surgery for arthritis and soft tissue abscess. All improved with therapy except for one death due to sepsis.

Conclusion: Lupus patients are prone to develop salmonella bacteremia and extra-intestinal collections even in absence of gastrointestinal symptoms.


  P89 Category: Systemic Lupus Erythematosus Top


Evaluation of clinical profile of Systemic Lupus Erythematosus

Anuj Singhal1 , Naveen Chawla2 , CS Mohanty3 , Hemant P4 ; Rheumatologist, Dept of Medicine, Path & Oncopath, ED & Undersea, Medicine, INHS Asvini, Mumbai

Objective : To evaluate the patterns and clinical characteristics of Systemic Lupus Erythematosus patients.

Methods: This was an observational cross-sectional study conducted at a tertiary care centre in Mumbai. Patients who were diagnosed with Systemic Lupus Erythematosus (SLE) were enrolled in the study from 01 Jan 16 to 31 July 16. After history & clinical examination these patients were subjected to various diagnostic procedures so as to confirm the diagnosis of SLE. The clinical and immunological profile of all these patients was mapped to have better understanding of the disease pattern.

Results: A total number of 11 SLE patients were enrolled with a mean age of 28.5 years. Gender distribution was found to be female preponderant (72.7%). Patients had involvement of various systems arthritis/arthralgia (64%), nephritis (45%), skin involvement/rashes (18%), adenitis (9%) and edema (9%) (Chart 1). The patients were positive for antibodies like ANA, Anti Sm, SSA, Anti RO etc (Chart 2). As a standard of care, all the patients were previously taking dual or triple therapy comprising of a steroid with one or two of immunosuppressant for a mean duration of 2.9 years. The disease activity of these patients was evaluated using SLEDAI (SLE disease activity index); the mean value was 5.64. Consequently, all these patients were given steroids with steroid sparing agents to which they showed good to moderate response.



Conclusions: The observations of present study suggest that SLE patients may present with a wide variety of manifestations. Vigilant evaluation is required to diagnose the disease and its clinical pattern. Steroids and other immuno-suppressants can play an important role in management of these patients.

Key words: Systemic Lupus Erythematosus, Immune-suppressants, SLEDAI


  P90 Category: Systemic Lupus Erythematosus Top


Prevalence of Low Bone Mineral Density In Indian Systemic Lupus Erythematosus Patients And Its Correlation With Carotid Atherosclerosis

Rasmi Ranjan Sahoo, Urmila Dhakad, Siddharth Kumar Das, Saumya Ranjan Tripathy, Durgesh Srivastav, Harikrishnan V; King George Medical University, Lucknow - 226003, India

Background: Osteoporosis and atherosclerosis are among the main causes of morbidity and mortality in both the general population and SLE patients. Though epidemiologic studies have consistently reported an inverse association between bone mineral density(BMD) and structural and functional measures of atherosclerosis in the general population, the data in SLE patients is lacking. As Indians are known to be metabolically predisposed to develop early atherosclerosis, it may be prudent to study a SLE cohort from this part of world for such an associa tion.

Methods:
26 confirmed SLE patients (fulfilling the 1997 ACR criteria) >18 years of age, were included in the study. Twenty-nine age-and sex-matched healthy controls were recruited from the same geographic area. Dual Energy X-ray Absorptiometry (DEXA: GE, Lunar) was used for estimation of BMD at three sites (proximal femur, lumbar spine (L1-L4), and distal radius) in g/cm2 in both patients and controls. Bilateral common carotid arteries were screened for patients using GE LOGIQ e ultrasound instrument. SLE disease activity index (SLEDAI) was also calculated.

Results:
Mean age of patients was 29.5+10.2 yrs whereas that of healthy controls was 27.7+5.03 yrs. All patients were females with a mean BMI of 22.4+4.47. The mean BMI of healthy controls was 22.95+2.62. Mean duration of disease among patients was 2.7+2.2 yrs. Low BMD (z score -2 or less) was seen in 26.9% of patients at L1-4 (vs 3.33% of controls, p: 0.001), 19.2% at radius 33% (vs none in controls) and 7.6% at neck of femur (vs none in controls). BMD at L1-4 and neck of femur did not correlate with CIMT, SLEDAI score, duration of disease and mean dose of steroid.

Conclusion:
Low BMD is more prevalent among SLE patients, yet it did not correlate with carotid intima-media thickness(CIMT), SLEDAI score, duration of disease and mean dose of steroid.


  P91 Category: Systemic Lupus Erythematosus Top


A clinical study of systemic lupus erythematosus (SLE) with special reference to complications both fatal & nonfatal : Experience at a tertiary care centre

G D Choudhury, A Kumar, A Hedge,V Vasdev; Army Hospital (R&R) Delhi, India

Background/Purpose: To evaluate the nature and complications of this disease in our patient population.

Methods: Patients diagnosed SLE on the basis of 2012 SLICC criteria attending the rheumatology services between 01 Oct 2014 to 30 Apr 2016 were included in the study. Data was collected from out patient follow up book and case records of admitted patients. Patients who were found to be only ANA positive but not fulfilling any other SLICC criteria were not included in the study.

Results: A total 151 adult and 2 juvenile lupus (all female) were included in the study. Median follow up was around 4.5 yrs with a range of 0.5-11 yrs. Various systemic and immunological complications encountered in these patients are as follows: Antiphospholipid syndrome(APS) in 48 (31.7%), Lupus nephritis in 96 (63.5%), Pulmonary Arterial Hypertension(PAH) in 08 (5%), Hemolytic anemia in 15(9.93%), Myositis in 12(7.94%),Neuropsychiatric SLE(NPSLE) in 18 (11.92%),Interstitial lung disease(ILD) in 13 (8.6 %),Macrophage activation syndrome 12 (7.94%). Few special cases need to be mentioned. A case of acute onset paraparesis with sensory loss and bladder and bowel involvement. She was ANA positive and also ant- NMO antibody positive with MRI showing longitudinally extending transverse. A case of SLE with right sided hemiparesis as initial manifestation. Another case of SLE with secondary APS developed hepatic vein thrombosis in spite of adequate anticoagulation. A few fatalities due to infections like CNS tuberculosis, macrophage activation syndrome and gastrointestinal vasculitis with gut ischemia occurred in spite of optimum therapy.

Conclusion: Systemic lupus erythematosus is an immunological disorder with multisystem involvement which at times poses a therapeutic challenge and diagnostic dilemma. Careful monitoring and early intensive intervention is the keystone to tackle its catastrophic complications


  P92 Category: Systemic Lupus Erythematosus Top


MELANONYCHIA-AN UNDER REPORTED CUTANEOUS MANIFESTATION OF SLE

Mythili Seetharaman, Rajeswari Sankaralingam, Tamilselvam Tiruchengode Natesan, Balameena Selvakumar, Saravanan Mayilsamy, Vignesh Mantharam, Sowndhariya V Annamalai, Sivakumar Vengudusamy, Balaji Chilukuri, Ramesh R; Madras Medical College, Chennai

Background:
Melanonychia is brown to black discolouration of nails. Melononychia is associated with many conditions like pregnancy,trauma, hyperthyroidism, cushing syndrome,malignancy and others.SLE has several mucocutaneous manifestation classified as Lupus specific and non-specific by Gilliam classification.

Aim: To study the association of melanonychia in SLE patients in our centre.

Methods: A Cross-sectional study in which 150 patients of SLE and 150 patients of rheumatoid arthritis were screened for presence of dyschromic nails -melanonychia in one or more digits.32 patients of SLE and 4 patients of rheumatoid arthritis had melanonychia. Prevalance of melanonychia in SLE(21.3) was more than prevalence in Rheumatoid arthritis(2.66).Hence association of melanonychia with various clinical manifestations of SLE and markers of disease activity like anti ds DNA,C3 C4 levels,ESR and CRP were studied by comparing 32 SLE patients with melanonychia and 32 patients without melanonychia.

Results: The 2 groups of SLE patients with and without melanonychia were comparable in age and sex. Mucocutaneous manifestations of SLE had significant association with the presence of melanonychia. (p value 0.00). association of melanonychia with other clinical manifestations like hematological(p value 0.8), NPSLE(p value 0.606), Lupus nephritis(p value 0.784), serositis(p value 0.554),Vasculitis(p value 0.32), musculoskeletal(p value 1.00 ),Pulmonary hypertension(p value 0.302) were not statistically significant.C3 levels (p value 0.599),C4 levels(p value 0.453),ESR(p value-0.6),CRP (p value 1.0) showed no significant difference between 2 groups.

Discussion: Melanonychia in patients with SLE has significant association with the mucocutaneous manifestations of SLE. This gives rise to a debate "Should melanonychia be included as non-specific skin lesion of Lupus?".


  P93 Category: Systemic Lupus Erythematosus Top


Successful treatment of Shrinking Lung Syndome in a case of Systemic Lupus erythematosus with Rituximab

Hegde Arun, MN Arjun, Vasdev Vivek, Kumar Abhishek, Kishore Kunal, Choudhury GD; Army Hospital Research & Referral,New Delhi

Background: Shrinking lung syndrome (SLS) is a rare and infrequently reported manifestation of systemic lupus erythematosus (SLE). The reported prevalence is around 0.5% in SLE patients. We report the case of a 34 year old male with SLS who was successfully treated with Rituximab.

Case Report:
This 34 years old male became symptomatic in May 2012 with symmetric inflammatory polyarthritis involving small joints of hands, wrist and elbows. He also had low grade fever lasting 15 days. Evaluation revealed a positive ANA (4+, speckled pattern) on immunofluroscence with Extractable nuclear antigen revealing a positive U1 Sn RNP, Ku & Ro52 Kd. Anti dsDNA titres were positive by ELISA. He was started on HCQS and low dose steroids with initial improvement in symptoms, however he stopped medication after 2 months and was lost to follow up. The patient again became symptomatic with easy fatiguability and breathlessness on exertion in Jan 2016. Evaluation for the same revealed elevated Rt hemidiaphragm on X ray Chest with USG Chest revealing reduced movement of medial aspect of Rt hemidiaphragm. HRCT chest done did not reveal any pleural or parenchymal disease. PFT showed moderate restriction with severe diffusion impairment. The pt was diagnosed as a case of SLS and started on high dose steroids. However he continued to be symptomatic with breathlessness and was exhibited Inj Rituximab 1 gm IV on day 0 and day 15. Follow up at 6 weeks post Rituximab revealed improvement in symptoms and PFT.

Conclusion:
The successful use of B cell depletion in a case of SLS in the background of SLE further goes on to highlight the ever expanding role of Rituximab in SLE. and to the best of our knowledge is the first case report from India


  P94 Category: Systemic Lupus Erythematosus Top


Rare And Potential Fatal Presentation of Lupus

Dr Manisha Ashwin Daware1 , Dr Pradeep Telli2 ; Narayana Health and Mazumdar Shaw Medical Centre, Bangalore (KA)

Background: Systemic lupus Erythematosus (SLE) is a multisystem autoimmune connective tissue disorder of unknown aetiology. CNS manifestation in lupus is seen in about 30-40% patients. Various manifestations like CNS or peripheral nervous system involvement have been described, sometimes in combination. Neuromyelitis optica (NMO) has been described in literature as case reports only.

Methods: 52 year old lady was admitted at intensive care unit with drowsiness, fever, arthritis, malar rash and myelitis of 2 months duration. She had hypothyroidism, bronchial asthma and old pulmonary Koch's (treated). On evaluations, she was found to have SLE with brain infarcts, musculoskeletal and haematological features (clinical laboratory and immunological criteria). She was managed with high doses of steroid (tapered later) and mycophenolate mofetil. Further in course she developed left optic neuritis for which cyclophosphamide was started. Within one month of completion of cyclophosphamide 6 pulses she suddenly developed quadriparesis secondary to extensive myelitis. Infection was ruled out after CSF analysis and cultures. NMO antibodies were positive in high titres. She was treated with steroid and plasmapheresis. She had reactivation of pulmonary Koch's during treatment and other secondary systemic infections.

Results:
She succumbed to sepsis despite combination of ATT, antibiotics and immunosuppressive treatment.

Conclusion:
NMO has been described as potential fatal complications in lupus despite combination of immunomodulators. Early diagnosis and treatment of the condition may carry better prognosis.


  P95 Category: Systemic Lupus Erythematosus Top


Myelitis in systemic lupus erythematosus does not always imply lupus activity: A case report.

Abhishek Kumar, Vivek Vasdev, GD Choudhury, Arun Hegde, Arjun MN ; Army Hospital (Research & Referral), Delhi cantt - 110010

Background: Patients with Systemic lupus erythematosus (SLE) or Sjogren syndrome (SS) often have been found to have Anti-aquaporin-4 antibodies (AQP-4IgG), which are strongly associated with neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD). It is important to define whether NMOSD and SLE/SS are independent entities that coexist with each other, or they comprise spectrum of the same disease, since this can have significant therapeutic and prognostic implications.

Case Summary:
This 27 year old lady presented with acute onset paraparesis, sensory loss, bowel and bladder incontinence without any other neurological deficit. There was no history suggestive of polyarthritis, or malar rash. As part of initial neurological evaluation, MRI spine revealed evidence of longitudinally extensive transverse myelitis (LETM) involving D4-D6 levels. Further investigations revealed anemia, elevated acute phase reactants, proteinuria and absence of active urinary sediment. CSF analysis showed raised leukocytes, mild increase in proteins and negative oligoclonal bands. Anti-nuclear antibody (ANA) by indirect immunofluorescence and anti-double stranded DNA antibody (dsDNA) were positive with normal serum complements. Antiphospholipid antibodies, serological markers of systemic vasculitis and neurotropic infections were also negative while anti- AQP-4 IgG was positive by immunofluorescence. Patient fulfilled criteria for both SLE and NMOSD. The choice of therapy for both conditions is different and presence of anti- AQP-4 IgG is predictive of debilitating disease. A careful literature review revealed that anti- AQP-4IgG are highly specific for NMOSD and any patient with LETM who test positive for this antibody, irrespective of coexistent SLE or SS should be treated as a case of NMOSD. The patient was managed with pulse steroids, followed by Rituximab with complete neurological recovery.

Conclusion : It is important to screen all patients with LETM for NMOSD and connective tissue disease. Myelitis in such a case should be treated as part of NMOSD.


  P96 Category: Systemic Lupus Erythematosus Top


Juvenile male SLE with predominant skin and oral manifestations

Anupam Dey; All India Institute of Medical Sciences Bhubaneswar, Odisha

Background/Purpose: Systemic lupus erythematosus (SLE) is predominantly a disorder of females. Males are rarely affected including adolescent males. Juvenile SLE is a more aggressive disease than adult SLE. This case highlights predominant skin and oral lesions in a juvenile SLE.

Case:
A 16 yrs old male presented with history of intermittent scattered skin lesions over scalp, ears, back, and lateral part of arm over 2 years and intermittent fever along with painful oral ulcers and flare of the skin lesions over extremities and trunk for 1 month. Both knee joints had intermittent pain. Skin showed scattered discoid lesions (pigmented raised border with central atrophy and depigmentation) (Fig 1). There was diffuse hair fall with atrophic scars. Ear lobes had atrophic scars. Hard palate had erosions (Fig 2) and the lower lip had a single erosion. Systemic examination was within normal limits. Hemoglobin was 8.8 gm%, TLC 3900/microL, and platelets were 2.87 lakhs. ANA and dsDNA were positive. Complement levels were low. Liver and kidney function tests were normal. The patient was treated as a case of Systemic Lupus Erythematosus (SLE) with steroids.



Discussion:
SLE is a multisystem disorder of autoimmune etiology characterized by autoantibodies against self-proteins. It is more common in females of child-bearing age with reported female:male ratio of 8 -15:1. Post puberty, the sex difference is about 4:1. Predominance in females may be the effect of endogenous sex hormones exerting their effects on the immune system. Hence male lupus is a rare phenomenon. It has been reported that male patients had more renal involvement, neurological involvement, thrombotic episodes, arthritis, and cardiovascular involvement. Male SLE patients are usually older at disease onset. Voulgari et al. found that men had significantly less photosensitivity, oral ulcers, thrombocytopenia or increased ESR in comparison with women.

Conclusion:
Juvenile males with SLE can present with predominantly skin and oral lesions.


  P97 Category: Systemic Lupus Erythematosus Top


"SLE THE GREAT IMITATORS" A case of young male presenting with seizures, diagnosed SLE and lupus nephritis.

Varun Bhatia; Government medical college, Surat

Background/Purpose: SLE is one of several diseases known as "the great imitators" as it often mimics or is mistaken for other illnesses. Case report of 14year male patient with h/o seizure, presented with bicytopenia and nephrotic syndrome diagnosed as SLE.

Methods: Case report 14year male patient presented with 5 episodes of generalised tonic clonic seizures, breathlessness for past 6 months. On examination hyperpigmented, nonpruritic rash in malar area, on both pinnas and on the back, ulcer over the hard palate, diffuse scalp alopecia. Lymphadenopathy was present in submandibular region. vitals were normal.On chest auscultation bilateral rhonchi present. Past/family medical history-not significant. Laboratory investigations : Hb 6.9 gm/dl, White blood count- 3700/cmm, platelets- 241000 /L,serum albumin- 2.0mg/dl, S. creatinine 0.8mg/dl. Urine routine- albumin 2+,24 hrs urine protein was 3.1gm. Lipid profile - triglycerides 1540mg/dl, cholesterol total 190mg/dl,cholesterol/ hdl ratio 7.9. ANA was positive with a dilution of 1:100(++++)à specked pattern. Anti Smith antibodies, anti ds dna and Ribosomal pàpositive(Blot). Kidney biopsy - grade 5 lupus nephritis (membranous GN). C3- 0.09 (gm/l). MRI brain -normal. Lymph node biopsy of submandibular region - chronic reactive hyperplasia.2D ECHO and Chest X Ray normal.

Results: Patient on presentation was worked up for seizure disorder (MRI brain- normal) treated with leviracetam. Lipemic serum on routine laboratory investigations was an incidental finding. With Hyperlipidemia (triglyceride- 1520mg/dl) the diagnosis of possible familial hypercholesterolemia was initially thought but later was concluded as part of Nephrotic syndrome.With Bicytopenia, nephrotic range proteinuria and ANA, anti-SM, antiDs DNA strong positivity, with elevated ESR and Hypocomplementemia lead to diagnosis of SLE.Kidney biopsy revealed grade 5 (membranous GN). Treatment with pulse methyl prednisolone, azathioprine, Hydroxychloroquine and hematinics was initiated.

Conclusion: Suspecting SLE by consolidating all clinical and laboratory parameters resulted in early diagnosis of CNS Lupus and Lupus nephritis.


  P98 Category: Sjogren's Syndrome Top


Serum Interleukin 17 In primary Sjogren's Syndrome: Is There A Difference Between Glandular And Extraglandular Manifestations?

Kavitha Mohanasundaram1, 3 , Bhuvanesh Mahendran,1 Saranya Chinnadurai1 , Balameena Selvakumar,1 Therese Mary D2 , Rajeswari Sankaralingam1 ;1 Institute of Rheumatology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai. Department of Rheumatology,2 Department of Immunology Madras Medical College,3 Saveetha Medical College, Thandalam

Background: Primary Sjogren's Syndrome(pSS) is a systemic disease with wide array of life threatening extra glandular manifestations. Recent studies point towards IL 17 A as a possible cytokine in its pathogenesis.

Aim: To study the levels of Sr.IL 17 with respect to glandular and extraglandular manifestations of pSS.

Materials and Methods:

Duration : March 2013 to August 2015.

Inclusion criteria
: Newly diagnosed pSS patients (revised AECG criteria of 2002)

No of subjects with predominantly glandular manifestations n 20

Pre dominantly extraglandular manifestations n 62

Exclusion criteria : Secondary Sjögren's Syndrome, Overlap Syndrome

Healthy Controls n = 20

They were subjected to baseline investigations and imaging as needed. All patients underwent Schirmer's Test and lip biopsy irrespective of sicca symptoms. Sr.IL 17 levels were done using ELISA methodology; anything below 3 pg/ml was considered negative.

Statistical Analysis: SPSS Software 20 version

Results:

Extraglandular manifestations of pSS in our study were arthritis, interstitial lung disease, cutaneous, renal and neurological.

A.
The mean Sr.IL 17 levels of healthy controls and pSS was 0.21±1.8 pg/ml and 7.92±22.46 pg/ml respectively(p value <0.01)

B. Sr.IL 17 levels within two groups of pSS

Mean Sr.IL 17 levels in Glandular group 13.03+29.01 pg/ml

Extraglandular group 6.274+19.0 pg/ml (p<0.05)

C. Analysis of Sr.IL 17 levels in the subgroups of pSS

Discussion:

  1. Sr.IL 17 was increased significantly in pSS compared to healthy controls
  2. Sr.IL 17 levels were significantly elevated in pSS with glandular, arthritis and renal manifestations and did not show any difference from healthy controls in the respiratory, neurological and cutaneous group.


Conclusion: IL 17 is elevated in glandular manifestations of pSS more than the extraglandular manifestations, probably the germinal centres of gland plays a major role in IL 17 production in pSS.


  P99 Category: Sjogren's Syndrome Top


Minor Salivary Gland (Msg) Biopsy Findings In Indian Patients With Sjögren's Syndrome And Their Correlation With Extra-Glandular Manifestations

Dr Sharath Kumar, Dr Divya Puttegowda, Dr Pournima Kamate, Dr Sanjay Pai; Columbia Asia Hospitals, Bangalore, Jawaharlal Nehru Medical College, Belgaum



Background/Purpose: There has been no data regarding biopsy findings and utility in Indian patients as well as it's correlation to clinical features.

Methods: Retrospective review of all minor salivary gland biopsies in our hospital from July 2013 to July 2015.Among the biopsy specimens, Sjögren patients slides were reviewed by pathologist.Clinical characteristics were determined by retrospective chart review.

Results: A cohort of 26 patients emerged. Nearly all were female (25/26) and fulfilled the ACR criteria (25/26.Twenty one patients had an extra-glandular manifestation.(Arthritis in 14 patients,2 had renal involvement 2 had vasculitis,3 had ILD,1 had PAH and hematological involvement was in 7 patients). MSG biopsy was positive in 20.The mean focus score was 1.7/mm2 with a maximum score of 9.57/mm2. There was no statistical difference in proportion of positivity of biopsy (4/5) or mean focus score (2.01/mm2 )between patients with extra-glandular involvement and those without(16/21 and 1.71/mm2 ). Only 2 patients (7.7%) had germinal centers (GC) like structures in the biopsy. Both patients with GC like structures had extra-glandular features, were biopsy positive and had a focus score of 6.175/mm2 . Among patients without GC like structures 19 of 24 had extra articular features, 18 of 24 were biopsy positive and the mean focus score was 1.41/mm2 .

Conclusion: In this small cohort of Indian Sjögren's patients who had undergone biopsy (similar to previous Indian series) extra-glandular features were more frequent than in western cohorts. Only two patients had germinal centers. (c.f.~25% in Theander et al.).Among patients with GC like structures there seemed to higher focus score and more extraglandular features.However, biopsy findings did not appear to differ between patients with extraglandular manifestations and those without.


  P100 Category- Sjogrens Syndrome Top


Do patients with Primary Sjogren's presenting early in disease have more extraglandular features ?

Pandya Sψ , Solanki R, Parikh T; Vedanta institute of medical sciences, Ahmedabad

Background/Purpose: With increasing awareness and better diagnostic facilities, more and more patients are being diagnosed primary Sjogren's syndrome (pSS) early in their disease. We wanted to study if these patients (< 2 years of symptoms) had more of extraglandular features.

Material and Methods: This was a cross sectional study. The study period was from 1st Jan 2014 to May 31st 2016. Informed consent and EC clearance was obtained. Data was extracted from our records. Patients fulfilled ACR-EULAR criteria for pSS. We used descriptive statistics to compare data between two groups - those early in disease (< 2 years of symptom onset) vs late in disease. We also compared our data to that form other series reported from Sweden, China and South India (Vellore).

Results: Patients early in their disease were significantly younger (mean 42.2 vs 47.4) than those presenting late. They also had significantly less musculoskeletal manifestations (36.5 vs 56.6%) which means musculoskeletal manifestations (arthralgias/arthritis/myositis) may be appearing later in disease. Rest of the demographic and immunological profile were not significantly different between the two groups. When we compared our overall data to that reported from other countries, we found decreased MSK manifestations in our series vs Swedish and South indian series, decreased Raynaud's phenomenon in our group vs Swedish and Chinese group but more than the South Indian group, decreased pulmonary manifestationsvs Chinese group, decreased renal involvement in ours vs Chinese and Swedish groups, Anti La positivity was significantly more (75 vs 40%) in our patients vs all other groups.

Conclusion: Except for musculoskeletal manifestations, which was less common in early disease, none of the other extraglandular manifestations were more in early SS patients vs those late in their disease. Anti La antibody was often positive in our group vs others.


  P101 Category- Sjogrens Syndrome Top


Vitamin D in Sjogren's syndrome: is it a different story?

Mashru Puneet1 , Pulukool Sandhya1 , Debashish Danda1 Gowri Mahasampath2 , Joseph Dian Bondu3 , Victoria Job; Department of Clinical immunology and Rheumatology, Department of Biostatistics, Department of Clinical Biochemistry, Christian Medical College and Hospital Vellore

Objective: The purpose of the study was to look for any association between 25-hydroxyvitamin D(25(OH)D) levels and disease manifestations in Indian patients with primary Sjogren's syndrome (pSS).

Methods: Patients satisfying the American European Consensus Group (AECG) or American College of Rheumatology (ACR) 2012 for pSS between 2008 and 2015 were included if baseline 25(OH)D levels were available in hospital's laboratory record. 25(OH)D sufficiency was defined as >30 ng/ml. Clinical, laboratory data and disease activity scoring by ESSDAI were retrieved retrospectively. Latitude corresponding to residence of each patient and the season of performing the assay were recorded. Chi-Square test was done to find relationship between the vitamin D deficiency and categorical variables. Odds ratio was calculated using univariate logistic regression with 25(OH)D as exposure.

Results: Mean 25(OH)D for 235 patients with pSS was 19.98(12.55). 25(OH)D deficiency, insufficiency and sufficiency was seen in 141(60%), 60(25.5%) and 34.0 (14.5%), respectively. No association was noted between season of performing assay and the levels. pSS with 25(OH)D ≤30ng/ml had more than two fold risk of higher grading on lip biopsy as well as RF positivity. However, low 25(OH)D seemed to be associated with lower ESSDAI and less pulmonary involvement.

Conclusion: Prevalence of 25(OH)D deficiency in Indian patients with pSS was comparable to that of general Indian population. 25(OH)D deficiency was associated with higher odds for RF positivity and positive grading on lip biopsy. Surprisingly, 25(OH)D deficiency was associated with lower ESSDAI score.


  P102 Category- Sjogrens Syndrome Top


Hypokalemic paralysis in primary Sjögren's syndrome- a single center case series (2015-2016)

Nahar Naisar, Nahar Prachi; Arham Rheumatology Center, Nasik, Maharashtra

Background/Purpose: Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by progressive lymphocyte infiltration of the exocrine glands. pSS has been associated with numerous extra glandular manifestations and has heterogeneous presentation. Although distal renal tubular acidosis (dRTA) is very common in pSS but it is usually asymptomatic and goes undetected in most of the cases. pSS with hypokalemic periodic paralysis (HPP) is increasingly reported in India. Present study evaluates HPP as presenting feature in pSS.

Methods:
Subjects of primary Sjögren's syndrome (pSS) were identified retrospectively from database maintained at our center. The diagnosis was clinical. Clinical and investigations data was extracted pertaining to initial examination and follow up. Standard investigations & ELISA, immunoblot and nephlometry to assay autoantibodies (AAb) were done

Results: 18 patients of primary Sjögren's syndrome (pSS) were identified in the period from June 2015 to August 2016. Out of 18 patients, 5 patients presented with hypokalemic periodic paralysis (HPP) as presenting feature to us. All were females with average age of 45 years. All patients had objective evidence of ocular sicca but only 40% were symptomatic. Oral sicca was reported by 40% patients. Other features include arthralgias (40%), skin rash (40%), cytopenias (20%). All 5 patients were positive for ANA (100%). SSA was positive in 100%, SSB in 40% of patients & Rheumatoid Factor in 60%.

Conclusion: The kidney involvement in pSS can uncommonly present as hypokalemic paralysis in the absence of significant sicca symptoms or may precede sicca symptoms. A high index of suspicion for pSS should be kept in all patients with hypokalemic paralysis. This phenotype may represent a distinct subset.


  P103 Category- Sjogrens Syndrome Top


Sjogren Syndrome with Hypokalemic Paralysis with High anion gap metablic acidosis - a newly encountered entity.

Abhishek Agrawal, Sharad Dev, N.K. Singh, Nilesh Kumar; Banaras Hindu University, Varanasi

Background: Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement. Renal tubular acidosis is a well known extraglandular manifestation of Sjogren Syndrome. Renal tubular acidosis is associated with hypokalemia but causes normal anion gap metabolic acidosis. But here we are presenting a case of Sjögren syndrome with suspected renal tubular acidosis with hypokalemia but having high anion gap metabolic acidosis.

Case Summary: A 60 years old male patient came with complains of mild grade fever for 10 days followed by acute onset rapidly progressive quadriparesis for 1 day without bladder bowel or sensory involvement. Laboratory features revealed severe hypokalemia with high anion gap metabolic acidosis. His serum creatinine was 1.7 mg/dl and urea was 67mg/dl. Urinary anion gap was normal. All possible causes of high anion gap metabolic acidosis were extensively ruled out. Immunological work up showed high titres of anti-Ro antibody and low titres of anti-La antibody. Histopathology of minor salivary gland showed lymphocytic sialadenitis. Patient improved on potassium and bicarbonate supplement.

Discussion: Renal involvement in Sjögren's syndrome is not uncommon and may precede sicca complaints. The pathology in most cases is a tubulointerstitial nephritis causing distal RTA, and, rarely, hypokalemic paralysis. The renal pathology in our patient was not completely understood since RTA causes normal anion gap metabolic acidosis. We could not find any similar case report of Sjogren Syndrome with hypokalemic paralysis with high anion gap metabolic acidosis.


  P104 Category - Sjogrens Syndrome Top


Serum Vascular Endothelial Growth Factor Levels As A Marker of Skin Fibrosis And Vascular Damage In Systemic Sclerosis

Saranya Chinnadurai, Rajeswari Sankaralingam, Bhuvanesh Mahendran, Balaji Chilukuri, Balameena Selvakumar; Madras Medical College, Chennai

Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular damage and excessive fibrosis in skin and internal organs. Potent proangiogenic mediator vascular endothelial growth factor-A (VEGF-A) is overexpressed in the skin and circulation of patients with SSc.

Objective:

  1. To compare the serum levels of Vascular Endothelial Growth Factor (VEGF-A) in systemic sclerosis patients with healthy population.
  2. To correlate the levels of Vascular Endothelial Growth Factor (VEGF-A) in systemic sclerosis patients with the severity of skin fibrosis and vascular damage.


Methods: Serum VEGF levels were measured in 55 SSc patients who fulfilled 2013 ACR/EULAR classification criteria for scleroderma and 30 healthy age and sex matched controls by ELISA. All patients underwent detailed clinical examination, echocardiography, pulmonary function tests and high resolution CT scan of lungs.

Results: Baseline characteristics of patients and controls were comparable. Mean serum VEGF in SSc patients was higher than in the controls (641.27 pg/ml vs 187.12 pg/ml respectively; p<0.00001). Serum VEGF levels correlated significantly with the higher modified Rodnan skin scores (mRSS) (r=0.7185; p<0.00001). SSc patients with pulmonary hypertension (n=26) showed significantly higher mean VEGF levels than those without pulmonary hypertension (n=29) (824.61pg/ml vs 476.89pg/ml; p<0.00001). Patients with interstitial lung disease (ILD) (n=21) had significantly higher mean VEGF levels when compared to those without ILD (n=34) (804.28 pg/ml vs 540.58 pg/ml; p=0.0005). Higher serum VEGF levels correlated with lower forced vital capacity (r= -0.6386; p<0.00001). Mean VEGF levels were higher in patients with digital ischemia (digital pitted scars, digital ulcers and gangrene) compared to those without ischemic changes (p<0.00001).



Conclusion: Serum VEGF levels were elevated in systemic sclerosis and they correlated with the severity of skin fibrosis and vascular damage.


  P105 Category- Scleroderma Top


Botulinum Toxin Type A In The Management Of Resistant Raynauds Phenomenon Secondary To Autoimmune Systemic Connective Tissue Diseases

Dr. S. Sriram, Dr. S. Rajeswari; Madras Medical College and RGGGH

Aim: The primary objective of our study was to assess the efficacy of botulinum toxin type A(botox) in the management of recalcitrant secondary Raynaud's phenomenon with respect to complete pain relief (patient reported) after botox administration.The secondary objectives were to study the healing of digital ulcer,improvement in tissue oxygenation,improvement in activities of daily living and prevention of further episodes.

Methods:
This pilot study involved 20 female patients with recalcitrant Raynaud's (increased frequency, duration and severity of attacks inspite of nifedipine, fluoxetine and sildenafil) secondary to systemic connective tissue diseases (scleroderma and mixed connective tissue disorder). 10 were allotted to group A which received 100 units of botox in both hands (10sites/ hand; 5 units/ site; only in affected fingers). Group B received placebo injection (saline). All patients were reviewed on day 1, 3, 7 and then monthly once for 3 months and the results were tabulated. Statistical methods were done using SPSS software.



Results:
3 patients in group A reported complete pain relief in 24 hours and the remaining in 72 hours. All patients in group A were pain free during the 3 months follow up. In group B, the mean pain free period with drugs and placebo was 1.15 months. At 3 months, patients in group A had a statistically significant improvement as compared to group B in visual analogue scale for pain (p=0.02) and oxygen saturation (p=0.04). Group A had an improvement in quick Disabilities of Arm, Shoulder and Hand(DASH) score, but was not statistically significant(p=0.34).

Conclusion:
Botulinum injection rapidly reduces the pain, increases the symptom free period, improves tissue oxygenation and aids in the healing of digital ulcer. It is a good alternative to surgery and can avoid polypharmacy.


  P106 Category- Scleroderma Top


Predictors of ILD and its progression in a cohort of Scleroderma patients from western India: A prospective observational cohort study

Yathish GC1 , Rohini Samant1 , Ashok. Mahashur2 , Taral Parikh1 , Parikshit Sagdeo1 , Girish Kakade;1 Rheumatology,2 Respiratory Medicine, P.D Hinduja Hospital and Medical Research Centre, Mumbai, India

Background: Pulmonary involvement, including both interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), has become the primary cause of Scleroderma-related death. Little data has been published from India regarding the progression of ILD and Pulmonary arterial hypertension in Scleroderma.

Methods
: Prospective observational study involving 68 patients. Patients were evaluated at 0, 3, 6, 9 and 12 months. Patient's demographic and clinical details were recorded at baseline. Other than routine tests, Anti-nuclear antibodies (ANA), Spirometry, diffusing capacity of lung for carbon monoxide (DLCO) measurement, high resolution computed tomography (HRCT) of thorax, 6-minute walk test and echocardiography were done.

Results : Study included 68 individuals with Scleroderma (according to the American College of Rheumatology classification criteria for SSc) with majority of cases being females n=64 (94.1%). ILD was seen in 53(77.9%) patients with a mean duration of 4.17 years. When we compared the mean change in FVC, DLCO and PAH from the time of onset of ILD/PAH to the values at last follow up, there was no significant change. ILD patients with UIP pattern on HRCT, showed a significant progression in terms of >10% fall in FVC as compared to NSIP subgroup. None of the baseline parameters were predictive of ILD and its progression. Older age predicted onset of PAH.

Conclusions:

  1. None of the studied baseline variables were predictive of ILD and its progression.
  2. Only age had statistically significant effect as a predictor for the occurrence of PAH.
  3. The demographic and clinical profile of our cohort was comparable to other studies from India and western population. Calcinosis and telangiectasia were less frequently seen as compared with western population.



  P107 Category- Scleroderma Top


5 Year Outcome of Patients With Interstitial Lung Disease In Systemic Sclerosis At A Tertiary Care Centre Hospital

Mythili Seetharaman, Rajeswari Sankaralingam, Tamilselvam Tiruchengode Natesan, Balameena Selvakumar, Saravanan Mayilsamy, Sivakumar Vengudusamy, Vignesh Mantharam, Bhuvanesh Mahendran, Saranya Chinnadurai; Madras Medical College, Chennai

Background: Pulmonary involvement, including both interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), has become the primary cause of Scleroderma-related death. Little data has been published from India regarding the progression of ILD and Pulmonary arterial hypertension in Scleroderma.

Methods:
Prospective observational study involving 68 patients. Patients were evaluated at 0, 3, 6, 9 and 12 months. Patient's demographic and clinical details were recorded at baseline. Other than routine tests, Anti-nuclear antibodies (ANA), Spirometry, diffusing capacity of lung for carbon monoxide (DLCO) measurement, high resolution computed tomography (HRCT) of thorax, 6-minute walk test and echocardiography were done.

Results:
Study included 68 individuals with Scleroderma (according to the American College of Rheumatology classification criteria for SSc) with majority of cases being females n=64(94.1%). ILD was seen in 53(77.9%) patients with a mean duration of 4.17 years. When we compared the mean change in FVC, DLCO and PAH from the time of onset of ILD/PAH to the values at last follow up, there was no significant change. ILD patients with UIP pattern on HRCT, showed a significant progression in terms of >10% fall in FVC as compared to NSIP subgroup. None of the baseline parameters were predictive of ILD and its progression. Older age predicted onset of PAH.

Conclusions


  1. None of the studied baseline variables were predictive of ILD and its progression.
  2. Only age had statistically significant effect as a predictor for the occurrence of PAH.
  3. The demographic and clinical profile of our cohort was comparable to other studies from India and western population. Calcinosis and telangiectasia were less frequently seen as compared with western population.



  P108 Category- Scleroderma Top


Systemic Sclerosis Sine Scleroderma - Clinical, Immunological Profile And Outcome From A Tertiary Care Centre

Balaji Chilukuri, Rajeswari Sankaralingam, Bhuvanesh Mahendran, Selvakumar Balameena, Ramesh Ramamoorthy, Saranya Chinnadurai, Sowndhariya Velu Annamalai, Vignesh Mantharam; Institute of Rheumatology, Madras Medical College & Rajiv Gandhi Government General Hospital,Chennai

Background: Systemic Sclerosis (SSc ) is a connective tissue disorder characterized by autoimmunity, vasculopathy and fibrosis. The hallmark of SSc is skin thickening. Systemic Sclerosis sine scleroderma (ssSSc) is a variant of SSc which shares visceral, serological, vascular manifestations with SSc but lacks skin thickening. ssSSc is rarely reported.We report clinical, immunological profile and outcome of ssSSc in a tertiary care center.

Method
: 213 SSc patients satisfying ACR/EULAR classification criteria were included in the study.The diagnosis of ssSSc was based on Poormoghim criteria: absent skin thickening, Raynaud's Phenomenon or peripheral vascular involvement, positive ANAs, one visceral organ involvement typical of SSc and absence of another defined connective tissue disease. Demographic, clinical and laboratory and imaging profile of these defined patients were analysed.

Results: Cross sectional analysis of 213 cases of SSc showed prevalence of 5.1% of ssSSc (11 cases). Dyspnoea and Raynaud's were the most common presenting complaints. Lung and gastrointenstinal involvement were the most common internal organs involved. Pulmonary hypertension was present in seven patients and interstitial lung disease in two patients. All 11 patients had oesophageal dysfunction both symptomatic (8) and asymptomatic (3).One patient had foot drop. Renal involvement was not observed in any of the patients. Anti centromere antibody was positive in six,anti-topoisomerase I in two and nucleolar pattern ANA in three patients. Three patients received immunosuppression- pulse cyclophosphamide therapy. None of the 11 cases developed skin thickening or worsened over a period of 4 years.

Conclusion: SSc with features of Raynauds phenomenon, anti-centromere antibody positivity, pulmonary hypertension and oesophageal dysfunction was the most common pattern observed in our cohort.We observed a good prognosis for ssSSc in our cohort.


  P109 Category- Sarcoidosis Top


Arthritis In Sarcoidosis - A Multi-Centre Study

Able L1 , Alok N2 , Amita A1 , Anupam W3 , Avinash J1 , Durga PM4 , Madhusmita M5 , Parasar G6 , Parshant A7 , Ram NM1 , Sapan P8 , Saumya RT3 , Shiva P9 , Vikas A1 , Vineeta A10 , Vineeta S11 , Vir SN4 , Vishnu VR12 , Yogesh PS;1 Clinical Immunology, SGPGIMS, Lucknow;2 Pulmonary Medicine, SGPGIMS, Lucknow,3 Rheumatology, KGMU, Lucknow,4 Clinical Immunology, JIPMER, Pondicherry,5 Pulmonary Medicine, JIPMER, Pondicherry6 IPGMER, Kolkata,7 Punjab Rheumatology Clinic, Ludhiana8 Rheumatology, Vedant Institute of Medical Sciences, Ahmedabad, India,9 Rheumatology, Apollo BGS Hospital, Mysore,10 Pathology, SGPGIMS, Lucknow, India11 Rheumatology, St John's Medical College, Bangalore12 Vizag Rheumatology and Immunology Centre, Vishakhapatnam13 Manipal Hospital, Bangalore, India

Introduction: Data on articular involvement in sarcoidosis is sparse, with prevalence varying from 2.6% to 51%. We present multicentric Indian data of sarcoidosis patients with articular manifestations.

Methods: Case records of patients presenting to ten rheumatology centres from 2005 to 2016 with sarcoidosis were retrospectively reviewed. Diagnosis of sarcoidosis was based on (1) non-caseating granulomas negative for acid-fast bacilli, fungal elements or foreign bodies in lymph nodes, liver, skin or exocrine glands (2) oligo- or polyarthritis with or without hilar adenopathy, erythema nodosum, uveitis or facial palsy. Joint involvement was assessed clinically, classified as acute or chronic depending on duration of symptoms lesser or greater than 6 months.

Results: A total of 103 patients with sarcoid arthritis were reviewed.



Among 54 patients of acute arthritis with follow up details, 45 had achieved complete response with NSAIDs.Additionally, steroid (44/79), methotrexate (19/79), HCQS (14/79) and azathioprine (4/79) were given for extra-articular manifestations.15/24 patients with chronic arthritis, at median follow-up of 2 years, were in complete remission with 14, 11 and 5 patients on steroids, methotrexate and hydroxychloroquine respectively. One patient with ILD died due to lung infection.

Conclusion: Ankle is most commonly involved in sarcoid arthritis. Wrist involvement is more common with chronic sarcoidosis. Acute oligoarthritis is the commonest presentation with excellent response to NSAIDs.


  P 110 Category- Sarcoidosis Top


Atypical presentation and novel mutation of a case of Blau Syndrome / Early Onset sarcoidosis

Panigrahy. B & Basu. S; LV Prasad Eye Institute, Bhubaneswar

Introduction:
Blau Syndrome/Early onset sarcoidosis is a rare Monogenic auto inflammatory syndrome Characterized by Arthritis, Uveitis & Dermatitis with gain of function mutation in NOD2 gene. We are presenting a 12 year girl child with atypical presentation like intermittent fever since the age of 3 years, late onset of Arthritis and a yet unreported new mutation.

Case Presentation: A 12 year old female child presented with sudden, painful defective vision in the right eye one week and decreased vision left eye 5 years. Ocular diagnosis was Right anterior uveitis withvitreous haemorrhage and Left chronic anterior Uveitis with complicated cataract. Gives history of Intermittent fever, skin rash since age of 3 years, Painful swelling of both knees 6 months.

No family history of similar problem. Almost normal milestone of growth. Mildly anemic, mild tender boggy swelling of both knees. No other abnormality except hepatosplenomegaly. ESR raised, Chest imaging, tuberculin test and ANA were negative. Iris node biopsy showed noncaseating granuloma lesions. DNA-sequencing of peripheral blood mononuclear cells revealed a previously unreported heterozygous mutation at position 667 (exon 4) of NOD2 gene. She was treated with oral steroids and methotrexate. Underwent vitrectomy with anterior chamber tap in right eye and cataract surgery in left eye. Finally her best corrected visual acuity was 20/30 and 20/25 in right and left eye respectively.

Conclusion: Blau's Syndrome / EOS is a rare monogenic autoinflammatory syndrome due to gain of funtion mutation in Nod2 gene. Ours case is associated with predominantly extended triad features like fever, late onset of arthritis & hepatosplenomegaly which may be the cause of delayed diagnosis by 9 years. Ours is a new mutation (not reported till date ) in the NACHT domain of

NOD2 gene.


  P 111 Category- Sarcoidosis Top


Isolated organ Sarcoidosis involving Hepatic, Lacrimal and Cardiac

Shiva Prasad B N, Ravi Puranik, Veunugopal A, Jayakumar P, Mahesh; Consultant Rheumatologist, Consultant Cardiologist, Consultant physician, Consultant Radiologist,Consultant Pathologist, Apollo BGS Hospital, Mysore

Sarcoidosis is a chronic multi-systemic granulomatous disease of unknown etiology; related to exaggerated cellular immunological reactions and is characterized by multiple occurrences of noncaseating epithelioid granulomas in several organs. We present case of Hepatic, Lacrimal and Cardiac sarcoidosis each.

Case 1: 65/F presented with abdominal discomfort was evaluated. She was found to have hepatomegaly with abnormal liver function test. Further evaluation ruled out other organ involvement. PET scan revealed diffuse metabolic activity in liver only. Liver biopsy revealed non caseating granulomatous lesion which were suggestive of Sarcoidosis. Patient responded to the treatment with steroids and Azathioprine. No recurrence of symptoms after 2 years of treatment.

Case 2 : 50/M presented with swelling in the right upper lid region with drooping of the eyelids. He was evaluated and found to have revealed ill defined thickening of the lacrimal gland on right side. Biopsy of the lacrimal gland revealed non caseating granuloma suggestive of sarcoidosis. He responded to treatment with steroids and Azathioprine. No recurrence of symptoms after 2 years of treatment.

Case 3 : 32/M presented with palpitations and breathlessness episodes for the last 6 months. On evaluation found to have ventricular tachyarrhythmias. Cardiac MRI revealed features of cardiac sarcoidosis. Patient was treated with Steroids and Azathioprine. He responded to the treatment and after one year of follow up there is no recurrence of symptoms with lesion resolved on repeat MRI.


  P 112 Category- Connective Tissue Disorder Top


IL 6 Levels In CTD Associated PHT And Its Correlation With Severity Of Pulmonary Hypertension

Sivakumar V, Rajeswari S, Balameena S, Saravanan M, Euphrasia latha, Saranya C, Bhuvanesh M, Sowndhariya V.A, Mythili S, Kumudha M; Madras Medical College, Chennai

Background: Pulmonary arterial hypertension may complicate diverse connective tissue diseases such as systemic sclerosis, SLE, MCTD, RA and is an important cause of morbidity and mortality in this context.IL-6 is one of the most important cytokines in the development of PAH. IL-6-STAT-BMPR2 is a crucial pathway contributed to the understanding of the pulmonary arterial remodelling leading to pulmonary hypertension.

Aim:
To establish the role of IL 6 in CTD associated PHT and its correlation with severity of pulmonary hypertension.

Methods:
IL-6 levels were estimated in sera (at the time of diagnosis) of 65 patients with CTD. Out of 65 patients, 40 patients have CTDs (SLE-20, SSc-15, MCTD-5) with pulmonary hypertension and remaining 25 patients (SLE-10, SSc-10, MCTD-5) have CTDs without pulmonary hypertension. Patients with PHT secondary to ILD in CTD were not recruited into the study group. Normal IL-6 levels for our population were estimated from 60 healthy volunteers (69.33±11.70).

Results:
IL 6 levels were significantly elevated in patients with CTD-PHT than in patients without PHT This significant elevation of IL-6 was also noted in individual diseases, SLE (mean±S.D in pg/ml 366.23±53.12 vs 316.44±47.25) p=0.016, systemic sclerosis (mean±S.D in pg/ml 329.05±23.94 vs 275.15±48.42) p=0.0013, MCTD (mean±S.D in pg/ml 324.04±50.03 vs 238.32±30.33) p=0.014 with and without pulmonary hypertension respectively. Levels of IL-6 correlated with severity of PHT as measured by mPAP and 6MWD. IL-6 levels had a highly significant positive correlation with mPAP (r=0.82; P<0.05) and significant negative correlation with 6MWD (r=0.69; p<0.05)

Conclusion: Serum IL-6 levels are elevated significantly in CTD patients with PHT and it plays a major role in pathogenesis of PHT. Thus targeting IL-6 can be one of the modalities of treatment in CTD associated PHT.


  P 113 Category- Vasculitis Top


Takayasu Arteritis - Enigmatic and Erratic - IL-6, TNF-α, hsCRP and Intima Media Thickness with Tocilizumab and Mycophenolate Mofetil. A Tertiary care center experience.

Rajeswari Sankaralingam, Bhuvanesh Mahendran, Balaji Chilukuri, Saranya Chinnadurai, Ramesh Ramamoorthy, Euphrasia Latha, Tamilselvam TN, Balameena S, Saravanan M, Therese Mary; Madras medical College, Chennai

Background: Takayasu Arteritis (TA) is enigmatic and erratic. Till date no definite outcome measures are available. An attempt has been made with Tocilizumab(TCZ) and Mycophenolate Mofetil (MMF) treatment with assessment of IL-6, TNF-α, hsCRP, CRP,and Intima Media Thickness(IMT) levels in this study.

Methods:
Thirteen (F:M-11:2) TA patients(recalcitrant severe disease, cardiac, strokes, thrombosis, eye involvement) were chosen for TCZ therapy and 12 (F:M-9:3) in the MMF arm (for at least 1 year). IL-6, TNFα, hsCRP(by ELISA) ESR, CRP and IMT were assessed at baseline and 6 months after treatment. 20 age and sex matched healthy controls were also included in the study.

Results:
Clinical response in TCZ group was dramatic and response to MMF remained stable. IL-6 levels were higher in the TCZ and MMF groups when compared to healthy controls (mean 97.01pg/ml, 214.06pg/ml respectively vs 69.3pg/ml, p<0.0001). TNF-α levels were higher in TCZ and MMF groups when compared with controls (mean 47.5pg/ml, 176.8pg/ml respectively vs 72.5pg/ml, p<0.0001). IL-6 levels increased after TCZ, but decreased after MMF. TNF-α levels increased in both groups after treatment showing a concordant relationship between IL-6 & TNF-α pathways.Mean IMT in TCZ & MMF groups at baseline and after 6 months were (p-0.07 & p-0.18 respectively). Mean hsCRP and IMT in both groups before and after treatment was not statistically significant although individual correlations with clinical improvement was significant.

Conclusion:
TA patients on TCZ showed dramatic clinical improvement and MMF patients remained stable. TCZ seems to be good alternative to MMF in severe cases with few side effects.


  P 114 Category- Vasculitis Top


Clinical profile of ANCA associated vasculitis based on PR3 and MPO serology: a single centre prospective study

Natasha Negalur, Gayatri Ekbote, Muzaffar Bindroo, Dhiren Raval, Vinay Singal, Naval Mendiratta, Shruti Bajad, Rajiva Gupta; Medanta- the Medicity, Gurgaon, Haryana

Background:
Classification of vasculitis, especially small vessel, is still not very clear. But with the discovery of ANCA, all rheumatologists divide small vessel vasculitis as ANCA positive or ANCA negative vasculitis. It is difficult to classify ANCA associated vasculitis as Granulomatosis Polyangitis, microscopic Polyangitis, Eosinophilic Granulomatosis Polyangitis and idiopathic Cresentic Glomerulonephritis in the traditional sense. We propose to classify AAV as PR3 or MRO related vasculitis. In this paper, we try to look at the clinical presentation based on serology.

Methods: We studied the clinical profile of our patients with ANCA associated vasculitis. A total of 41 patients were included in our study from August 2015 till August 2016. Patients were classified according to PR3 and MPO serology [based on ELISA] and c-ANCA and p-ANCA [by IIF]. This study has been done after approval and ethical clearance from IRB.

Results: All the patients who were c-ANCA by IIF were found to be PR3 by ELISA and those who were p-ANCA by IIF were MPO by ELISA.



Conclusion: We find that classification of patients of AAV as PR3 and MPO is a much simpler and easier method. We also found that difference between upper respiratory tract involvement in PR3 and MPO group was statistically significant. The difference between other organ involvement in both groups was not statistically significant.


  P115 Category: Vasculitis Top


Roflumilast In Refractory Mucocutaneous Manifestations of Behcets Disease: Case Series

Paul Antony T, Antony T P1 , Jayaraj K1 ; Departments of Immunology,1 General Medicine, Amala Institute of Medical Sciences, Thrissur

Background/Purpose: Oral ulcers, the hallmark of Behηet's syndrome, can be resistant to conventional treatment; therefore, alternative agents are needed. Apremilast is an oral phosphodiesterase-4 inhibitor that modulates several inflammatory pathways and underwent a phase 2 study with good results. Unfortunately it is unavailable in India. Roflumilast is PDE4 inhibitor licensed for chronic obstructive pulmonary disease with similar pharmacologic properties.

Methods: Patients were eligible for the study if they met the criteria of the International Study Group for Behηet'sDisease,were at least 18 years of age, and failed on common conventional treatment. After informed consent patient was started on Roflumilast at 500ug daily for one week with subsequent reduction of the dose to 500ug on alternate days.

Results: We could enrol around ten patients. The mean followup was four monthsAll the patients had received colchicine, dapsone, topical steroids, methotrexate and even systemic steroids. Three patients had also received etanercept. All patients had complete resolution of oral ulcers by day 7. After reducing the dose to alternate day none had recurrence of the symptoms. The Roflumilast was continued for another three weeks and stopped. In three cases there was recurrence of the lesions. Most patients tolerated the drug. In three cases doses had to be reduced Initially due to gastric intolerance. In one case there was significant weight loss necessitating drug withdrawal

Conclusion : Roflumilast was effective in treating oral ulcers, which are the cardinal manifestation of Behηet's syndrome. This preliminary study was neither large enough nor long enough to assess long-term efficacy, the effect on other manifestations of Behηet's syndrome, or the risk of uncommon serious adverse events.


  P116 Category: Vasculitis Top


Henoch-Schonlein purpura in adults: Renal outcomes and differences from pediatric HSP

Vikas Gupta, Amita Aggarwal, Ranjan Gupta, Vikas Agarwal, Able Lawrence, Ramnath Misra; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow

Background/Purpose: Henoch-Schönlein purpura (HSP), is predominantly a disease of the childhood though it can rarely occur in adults. In adults, it is believed to be more severe. Thus, we studied the clinical parameters and the renal outcomes in adult-onset HSP (≥18 years) and compared them with childhood-onset HSP (≤18 years).

Methods: Case records of patients diagnosed with HSP in last 25 years in our department were retrieved. Data on clinical features, lab abnormalities and outcome was extracted.

Results: A total of 74 patients (22 adults, 52 children) were seen. Compared to children, most of the adults had purpura as the first clinical manifestation (86.3% vs 53.8%, p < 0.01) whereas abdominal pain was only rarely the initial symptom in adults (9.1% vs 38.5%, p < 0.02). Adults had a higher frequency of joint involvement (90.9% vs 40.4%, p < 0.001) whereas the frequency of renal (68.1% vs 48.1%, p = NS) and gastrointestinal involvement (72.7% vs 84.6%, p = NS) was similar to that in children. 15 adults (68.1%) had renal involvement, including 9 with non-nephrotic proteinuria, 4 with nephrotic-range proteinuria and 2 with isolated hematuria. 5 adults underwent renal biopsy, 3 of which had mesangioproliferative glomerulonephritis (ISKDC grade II) and one patient each had ISKDC grade I and grade IIIa nephritis. On median follow-up of 2 years, 10 achieved clinical remission whereas 2 had persistent proteinuria (at one year and until last follow-up). 3 patients were lost to follow-up. In comparison, 25 children (48.1%) had renal involvement, including 15 with non-nephrotic proteinuria, 6 with nephrotic-range proteinuria and 4 with isolated hematuria.

Conclusion: In contrast to children, adults with HSP rarely have abdominal pain as the first clinical symptom while purpura is the most common initial symptom in them. Adults with HSP nephritis have good renal outcome.


  P117 Category: Vasculitis Top


B Cell therapy in ANCA associated vasculitis- A single centre prospective observational study

Gayatri Ekbote, Natasha Negalur, Muzaffar Bindroo, Dhiren Raval, Vinay Singal, Naval Mendiratta, Shruti Bajad, Rajiva Gupta; Division of Rheumatology and Clinical Immunology,Medanta- the Medicity, Gurgaon, Haryana

Background: There are limited treatment options in ANCA associated vasculitis (AAV) who are refractory or who relapse on Cyclophosphamide. Recently with B cell therapy, outcome in these patients has improved. In this paper, we have discussed our single centre experience in treatment with B cell therapy for relapse and refractory cases.

Methods:
Patients diagnosed as AAV who had not achieved remission on Cyclophosphamide or had multiple relapses in spite of Cyclophosphamide were included in the study. They were given 1 gram of Rituximab (Rtx) on day 1 and day 15 as induction therapy and subsequently 6 monthly maintenance doses of 500 mg. Low dose steroids were given as co-therapy. No other immunosuppressive therapy was given.

The study was approved by IRB and given ethical clearance in December 2015.

Results:



Among the patients who received Rituximab, all had anti PR3 antibody positive. All of these patients fulfilled the criteria for Granulomatosis with Polyangitis (GPA). Indication for receiving Rituximab due to involvement of lung, renal, upper respiratory and nervous system was seen in 4 (60%), 1 (10%), 1 (10%) and 2 (20%) respectively & 2 (20%) patients were given Rituximab for persistent disease activity. Complications Only 1 patient had infusion reaction at the end of 1st induction dose. But she remained in remission after 1st dose.

Conclusion: In our experience, Rtx is a very good treatment option for relapse/ refractory cases of AAV


  P118 Category: Vasculitis Top


Role of Co-trimoxazole prophylaxis in ANCA associated vasculitis to prevent relapse

Gayatri Ekbote, Natasha Negalur, Muzaffar Bindroo, Dhiren Raval, Vinay Singal, Naval Mendiratta, Shruti Bajad, Rajiva Gupta; Medanta- the Medicity, Gurgaon. Haryana

Background: Frequent bacterial infections are proposed as a cause of relapse in ANCA associated vasculitis (AAV). To prevent this, Rheumatologists have been using Co-trimoxazole prophylaxis in AAV especially in non renal presentation of the disease. In our study, we have compared patients who have received co-trimoxazole prophylaxis with those who have not.

Methods:
All patients diagnosed as AAV based on ACR criteria and ANCA serology (done by IFA and ELISA) were included in the study. Their demographic, clinical and laboratory parameters were recorded. All patients who received co-trimoxazole fulfilled the criteria for Granulomatosis with Polyangitis (GPA). Data of patients who received and did not receive Co-trimoxazole were compared. This study has been done after approval and ethical clearance from IRB. Total 23 patients out of the cohort of 41 patients received co-trimoxazole antibiotic prophylaxis.

Results:



Conclusion: In our study, we have not found any beneficial effect of Co-trimoxazole as a prevention of relapse, but we need to do further studies for the same in a larger cohort.

However there may be a bias in the study as we gave co-trimoxazole prophylaxis only in patients who had upper respiratory tract or lower respiratory tract involvement.

Moreover, all patients received cyclophosphamide induction therapy and more patients needed to be given Rtx induction therapy in co-trimoxazole group as it had more relapses and refractory cases.


  P119 Category: Vasculitis Top


Outcome of Renal and non renal ANCA associated vasculitis- Single centre prospective study

Natasha Negalur, Gayatri Ekbote, Muzaffar Bindroo, Dhiren Raval, Vinay Singal, Naval Mendiratta, Shruti Bajad, Rajiva Gupta; Medanta- the Medicity, Gurgaon, Haryana

Background: Studies have found that disease behavior of ANCA associated vasculitis (AAV) differs in renal and non renal involvement and the disease shows more relapses in those with non renal type of disease. In our study, we have described the clinical and laboratory parameters as well as the outcome of those with renal and non renal AAV.

Methods:
This is an observational, prospective, single centre study of ANCA associated vasculitis. A total of 41 patients from August 2015 till August 2016 of proven ANCA associated vasculitis were included in the study. Patients were studied according to clinical profile, ANCA Serology (IIF and/or ELISA) and, wherever possible, by biopsy.

This study has been done after approval and ethical clearance from IRB

Results:



Conclusion : No significant difference in the outcome between the two groups in our study. However, larger cohort is needed for further comparison.


  P120 Category: Vasculitis Top


Electrocardiographic Analysis of Repolarization Changes in South Indian Children with Kawasaki Disease after the Acute Phase of Illness.

Reddy Siddarth, Rai Maneesh, Kamath Nutan; Kasturba Medical College, Mangalore, Manipal University, India.

Background/Purpose: Kawasaki disease (KD) may cause coronary artery involvement and myocardial dysfunction. Repolarization abnormalities like QT prolongation, QT dispersion (QTd), T wave peak to end (Tp-Te) interval are associated with increased risk of myocardial ischaemia, ventricular arrhythmias and sudden cardiac events. We undertook this study to find out if repolarization abnormalities are present in KD children without cardiac involvement during acute illness.

Methods: Twelve lead ECG obtained from twenty children with KD at least six months in the convalescent phase, twenty age and sex matched controls and 19 children of the study group after 9 ± 2 months was analysed. Measurements (QTd, (Tp-Te) interval, Tp-Te/QT ratio) were made using Aerospace digital standard calipers. Statistical analysis using student t test and analysis of variance done with SSPS version 17.0

Results: Mean value of QTd in first ECG in cases was significantly higher: 43.15 ± 14.13 versus 29.47 ± 8.637 in controls p= 0.001. Follow up ECG in 19 cases: mean value 46.26 ± 16.254 versus 43.89 ± 14,529 of these cases six months prior. p= 0.440. Qtd increased in follow up ECG but not of statistical significance. There was no statistical significance in the Tp-Te interval in Lead II (68.08 ± 17.11 versus 64.97 ± 14.185; p= 0.099) and Lead V5 (70.21 ± 15.293 versus 63.37 ± 10.877; p=0.509) and Tp-Te/QT ratio in cases compared to controls(Lead II- 0.21 ± 0.05 versus 0.21 ± 0.034; p=0.138; Lead V5- 0.22 ± 0.05 versus 0.20 ± 0.031; p= 0.734 and in the follow up of cases(19)- Lead II (0.21 ± 0.05 versus 0.20 ± 0.034; p=0.153) Lead V5 (0.22 ± 0.038 versus 0.20 ± 0.036; p=0.111)

Conclusion: Significant increase of Qtd in cases indicate myocardial repolarization changes in the absence of clinical carditis. This cohort may be at increased risk for developing ventricular arrhythmia.


  P121 Category: Vasculitis Top


Adult IgA Vasculitis: A retrospective case series based analysis of phenotypes

Chengappa K G, Emmanual Dantis, Jain Ankit, Seth Gaurav, Belani Pooja, K C Shanoj, Durga prasanna misra, Negi VS; JIPMER, Pondicherry

Background/Purpose: IgA vasculitis in adults is a relatively uncommon entity, which is generally missed when the manifestations are mild. Adults can have severe gastrointestinal and cardiac manifestations, along with frequent relapses. There has been no concrete attempt to look into the clinical course of IgA vasculitis in Indian context. This is an attempt to consolidate the data on adult IgA vasculitis that may help to better understand the disease.

Methods:
Departmental records of patients with IgA vasculitis diagnosed between 2008 and 2016 were retrospectively analysed. Patients satisfying the EULAR/PRINTO/PRES IgA vasculitis classification criteria were included. Clinical, demographic and treatment parameters were analysed.

Results: Twenty two patients (12 males and 10 females) were included. The average age at symptom onset was 33.9 +14.7 years. The commonest presenting symptom was palpable purpura, present in all patients. Twelve patients (54.5%) had gastrointestinal involvement at disease onset. Two patients had refractory GI vasculitis resistant to IV pulse Methylprednisolone, mandating plasmapheresis. Three patients had renal involvement and none have progressed to Chronic Kidney disease. The average BVAS3 at presentation was 3.18 + 1.25.

At follow up, 11/22 (50%) patients relapsed. Four had cutaneous, 3 had arthritis, 3 had gastrointestinal (GI) and 1 patient had both GI and skin relapse. One patient had a lower limb gangrene requiring amputation at the MTP joint. The baseline mean BVAS3 was same in relapsers and non relapsers (3.18 v/s 3.18).The average age at disease onset also was similar these two groups (34.18 + 15.38 and 33.63 + 14.74 years).

Conclusion: IgA vasculits in adults can have life-threatening GI and renal manifestations. Relapse occured in majority of patients. Palpable purpura and GI vasculitis were most common manifestations during relapse. No clinical or demographic predictors of relapses could be identified.


  P122 Category: Scleroderma Top


Survey of current practice of Scleroderma management in India

Yathish GC1 Ramesh Jois2 , Dharmanand BG3 , Vinod Ravindran4 ;1 Columbia Asia Hospital, Bengaluru, Karnataka,2 Fortis hospital, Bengaluru, Karnataka,3 Sakra World hospital, Bengaluru, Karnataka,4 Centre for Rheumatology, Calicut, Kerala

Background: Systemic sclerosis (SSc, scleroderma) is a rare disorder characterized by fibrosis of skin and visceral organs. Due to rarity of the disease, absence of new treatment guidelines and the popular perception of a difficult to treat disease, there is wide variation in the clinical practice across geographic regions.

Methods:
A questionnaire based cross-sectional survey was carried out during the International Scleroderma Symposium held at Bangalore on 16th and 17th July 2016.

Results:
Of the 290 delegates who attended the Symposium, 116 responded (response rate: 40%); and a majority were rheumatologists (87%). For diagnostic purpose, only 15(13%) used nail fold capillaroscopy and only 52(45%) used skin scoring routinely in their clinics. Majority (> 80%) used X- ray chest, Pulmonary function tests(PFT) and 2D ECHO routinely to check for organ involvement. Ninety-five % respondents admitted that they are still dependent only on 2D ECHO for the diagnosis of pulmonary hypertension. Eighty-six physicians (74%) felt that cardiologists are not keen on doing right heart catheterization (RHC) and is the major hurdle in getting a RHC done. Regarding the management of ILD, 100(86%) respondents were doing yearly PFT's to look for lung involvement, however 66 (57%) also did HRCT when ILD was clinically suspected. Sixty-three (55%) respondents preferred Cyclophosphamide for the managing ILD. Sixty-four (55%) had used biological agents in managing difficult SSc patients with Rituximab being the most common agent used for resistant ILD cases. Majority (43%) felt that lack of awareness leading to delayed presentation and variable affordability and access to care were the major hurdles in the effective management of SSc patients.

Conclusion:
This survey highlights wide variability in the current SSc management practice in India and also underscores the need for specific guidelines for the management of SSc in low income countries such as India.


  P123 Category: Scleroderma Top


Correlation of focal score in minor salivary gland biopsy and antibody positivity with clinical subsets in sjogrens syndrome-is it significant

Kavya Devi N1 , Vara Prasad IR2 , Liza Rajashekar3 ; Department of Rheumatology, Nizam's Institute of Medical Sciences.

Objective: To characterise the clinical features of patients with Sjogrens syndrome (SS) and their association with immunological and histopathological profile in our cohort.

Methods:
Medical records of patients with sjogrens syndrome reaching tertiary care hospital between May 2015 to May 2016 are reviewed and those patients fulfilling American European Consensus group (AECG) 2002 or American College of Rheumatology (ACR) 2012 classification criteria were included. 56 cases are categorised into two groups based on their organ involvement. Group 1includes those with renal, central or peripheral nervous system, cutaneous vasculitis and pulmonary involvement. Group 2 includes those with sicca, Polyarthralgias/polyarthritis, constitutional, salivary gland enlargement. These two groups are compared with their autoantibody status-anti-Ro/La [positive/negative] and focal score (FS) [FS ≥3 /FS<3] using fishers exact test. p value <0.05 is considered statistically significant.

Results: 56 cases (55 females and 1 male case) are included. Most common presenting clinical feature being Polyarthralgias/polyarthritis (22 cases) followed by sicca (21cases), hypokalaemic paralysis (12cases), constitutional symptoms (9 cases), neuropathy (6 cases) and 3 cases each of parotid swellings and mucocutaneous rash. Overall during the course of disease 48 members have oral sicca among which 32 cases required treatment, 32 members have ocular sicca of which 20 cases required treatment, polyarthritis/Polyarthralgias seen in 33 cases, constitutional symptoms in 18 cases, renal involvement in 15 cases, mucocutaneous rash in 11 cases, cutaneous vasculitis in 5 cases, neuropathy in 7 cases, salivary gland enlargement in 5 cases and interstitial lung disease in 1 case. Anti-Ro/La positivity is seen in 26 cases and both ANA and IgM RF positivity in 19 cases, only ANA positivity is seen in 45 cases. Proportion of patients with antibody positivity in group 1 are statistically significant (p<0.05) compared to group 2 whereas focal scoring is not.

Conclusion: Patients with sjogrens syndrome with antibody positivity are more likely to have internal organ involvement than those with antibody negativity whereas focal scoring has no bearing on it.


  P124 Category: Scleroderma Top


Serum CXCL4 Makes Its Mark !-As A Biomarker of Disease Activity In Systemic Sclerosis

Balaji Chilukuri, Rajeswari Sankaralingam, Saranya Chinnadurai, Tiruchengode Natesan Tamilselvam, Bhuvanesh Mahendran,Ramesh Ramamoorthy, Sowndhariya Velu Annamalai, Vignesh Mantharam; Institute of Rheumatology, Madras Medical College,Chennai

Background: Systemic sclerosis (SSc) is a unique disorder characterised by autoimmunity, vasculopathy and fibrosis.There is no promosing biomarker of disease activity identified so far to assess the multisystem clinical associations.CXCL4 (chemokine ligand 4) is an antiangiogenic and profibrotic cytokine protein secreted by plasmacytoid dendritic cells which are upregulated in SSc.

Aim:
Aim of this study was to explore the role of CXCL4 as a potential biomarker of disease activity in SSc.

Methods:
55 cases of SSc who satisfied ACR/EULAR 2013 classification criteria and 25 age and sex matched healthy controls were included in this study.Demographic, Clinical, laboratory and immunological parameters were recorded.CXCL4 levels (ng/ml) were estimated by ELISA method and results analysed by SPSS.

Results:
Baseline characteristics were comparable in both cases and controls.CXCL4 levels were high in SSc patients in comparison to controls (mean 57.2ng/ml vs 19.6ng/ml; p= 0.02). CXCL4 levels were higher in diffuse cutaneous SSc in comparison to limited cutaneous SSc (mean 77.8ng/ml vs :28.6ng/ml,p=0.004).SSc patients with ILD had higher CXCL4 levels than in those who did not have ILD (mean 88.44ng/ml vs 31.23ng/ml,p=0.001).CXCL4 levels inversely correlated with FVC% predicted (p=0.003). CXCL4 levels were higher in patients with higher mRSS-modified Rodnans Skin Score (p<0.001).CXCL4 levels were found to be higher in pulmonary hypertension associated with diffuse cutaneous SSc patients than limited cutaneous SSc (mean 128.4ng/ml vs 29.4ng/ml; p= 0.001).Anti-topoisomerase I was the most common autoantibody associated with high CXCL4 levels. CXCL4 levels showed no correlation with acute phase reactants - ESR and CRP.

Conclusion:
CXCL4 levels serve as a potential biomarker of disease activity in SSc. Higher CXCL4 levels are found in diffuse cutaneous SSc and SSc associated ILD. Higher CXCL4 levels correlated with high mRSS and low FVC% predicted.


  P125 Category: Systemic Lupus Erythematosus Top


A study of MCP1 (Monocyte Chemotactic Protein 1) and NGAL (Neutrophil Gelatinase Associated Lipocalin) as urinary biomarkers in Lupus Nephritis (LN)

Ramesh Ramamoorthy, Rajeswari Shankaralingam, Balaji Chilukuri, Tiruchengode Natesan Tamilselvam, Selvakumar Balameena, Sowndhariya Velu Annamalai, Vignesh Mantharam; Institute Of Rheumatology, Madras Medical College, Chennai - 03.

Background: The debate regarding the necessity of renal biopsy in lupus nephritis has been for years. A reliable marker which could clearly delineate the presence or absence of renal involvement and which serves prognostically has been searched till date which could avoid the cumbersome renal biopsy. We intended to study 2 such markers in lupus nephritis.

Aim:
To study the correlation between urinary biomarkers MCP1 and NGAL and the disease activity in lupus nephritis.

Materials and Methods:
After obtaining Institutional ethical clearance and written informed consent from patients, a prospective observational study was conducted among 40 Systemic Lupus Erythematosus(SLE) patients attending lupus clinic in a tertiary care centre. Urinary MCP1(uMCP1) and NGAL(uNGAL) were measured by ELISA on spot urine samples in 22 patients with active LN (at baseline and after 6months of cyclophosphamide pulse and 18 with out renal involvement. 20 age and sex matched controls was taken. Disease activity was measured using renal SLEDAI.Statistical analysis was done using SPSS software.

Results:
In patients with active LN, both uMCP1/Cr (mean - 92.78 pg/ml) and uNGAL/Cr (76.11 pg/ml) were significantly elevated (p<0.001). In patients with remission (after 6months of cyclophosphamide),there was a significant reduction in uMCP1/Cr (44.18pg/ml) and uNGAL/Cr (38.45pg/ml) (p<0.005). In both control group and SLE without renal involvement the values of uMCP1/Cr and uNGAL/Cr were normal (24.44, 22.22 pg/ml in controls and 24.3, 22.80 pg/ml in SLE without renal involvement).Values of uMCP1/Cr and uNGAL/Cr had a direct correlation with mean rSLEDAI scores. ; Conclusion : Levels of urinary biomarkers MCP1 and NGAL were elevated in active lupus nephritis and were significantly reduced after immunosuppression. They also had a positive correlation with rSLEDAI scores. uMCP1 and uNGAL may serve as alternative to renal biopsy and may help prognostically .


  P126 Category: Rheumatoid Arthritis Top


51 cases of Systemic Rheumatoid Vasculitis: Single Centre Experience

Sakir Ahmed1 , Amita Aggarwal1 , Able Lawrence1 , Vikas Agarwal1 , Ramnath Misra1 ; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute, Lucknow

Background/Purpose : Though Rheumatoid arthritis (RA)affects nearly 1% of adult population, Systemic Rheumatoid Vasculitis (SRV) is a rare manifestation.Withbetter control of RA, it is felt that the prevalence of SRV has come down.

Methods : All patients diagnosed with SRV during the last 27 years (1990-2016) were included and data was extracted from their case records and hospital information system.

Results : Fifty-one patients with SRV were seen during this period. The mean age (±SD) of patients was 52.2 (±11.8) years. Thirty-seven were females. Mean duration (±SD) of RA was 9.6 (±6.6) years. The common vasculitic manifestations included:mononeuritis multiplex in 26, non-healing ulcers in 24, palpable purpura and gangrene in sevenpatients each. Forty-four of these patients were Rheumatoid factor positive. Leucocytoisis and thrombocytosis were present in 19 and six patients respectively. Complement levels were available for 23 patients:C4 was low in eight but C3 was low only in one. Vasculitis was proven by biopsy in 20 patients. Other complication of RA in these patients included joint deformities in 26, subcutaneous nodules in 11,interstitial lung disease in seven, andaltantoaxial dislocation in five. All patients received steroids; 22 received cyclophosphamide, eight received intramuscular gold and four Rituximab. Follow-up data was available for 32 patients. Ulcers responded well. One patient needed forefoot amputation. Neurological deficits persisted in seven patients while six recovered completely. There were three deaths within six months of vasculitis.

Conclusion: SRV is still being found in India. Delay in diagnosis results in permanent neurological deficits and even mortality.


  P127 Category: Rheumatoid Arthritis Top


Does the anti CCP antibody confer a palindromic phenotype to rheumatoid arthritis ?

Pandya S,Solanki R, Parikh T; Vedanta institute of medical sciences, Ahmedabad

Background/purpose : While anti CCP antibody is known to cause an erosive RA, we were finding a lot of palindromic rheumatism (PR) patients also had CCP positivity. We therefore decided to study if anti CCP antibody was more often positive in PR as compared to RA.

Material and Methods : This was a cross sectional study. Study period was from 1st Jan 2014 to May 31st 2016. informed consent and EC clearance was obtained. Descriptive statistics was applied to the data we had on patients with PR and RA to look at differences in demographic profiles and autoantibody positivity (RF and CCP) between the two. We also compared our data to a series reported from Spain and Iran.

Results: A total of 297 patients with palindromic rheumatism presented during the period vs 1574 who fulfilled ACR 2010 RA criteria. There were siginificantly more (28%) males in the PR group than (15%) in RA. Mean age of PR patients was significantly lower (43.2 vs 47.4). They were also significantly early in their disease (3.2 years vs 6.3 years). While PR patients had significantly lower ESRs (40.4 vs 61.6), the difference between the two groups (PRvs RA) in terms of RF positivity (76 vs 73.6%) and CCP positivity (85.9 vs 74.5, p = 0.079) was insignificant. PR patients weighed significantly more than RA patients. When we compared our data to a series from Spain and Iran, significantly more PR patients in our group were females, older in age and had RF positivity vs Iran group AND significantly more were early in disease and had CCP positivity vs the Spanish group.

Conclusion: While CCP positivity was not positive in significantly greater proportion of our patients compared to RA, it was significantly higher in our PR group vs another group from Spain.


  P128 Category: Rheumatoid Arthritis Top


Formulation of Nano Lipid Vesicles For The Treatment Of Rheumatoid Arthritis

Avril Mathias1 ,Lenson Dsouza2 , Narayana Charyulu1; N.G.S.M. Institute of Pharmaceutical Sciences, Paneer, Deralakatte, Mangalore, Meditek India, Managlore

Purpose : The purpose of the study was to formulate and evaluate nano lipid vesicles to treat rheumatoid arthritis.

Methods : In this study, a comparison was made between thin film hydration technique and ether injection methods of preparation of nano lipid vesicles. In thin film hydration technique, lipids like DSPC, DPPG and cholesterol were dissolved individually in chloroform and methanol mixture and rotated in the rotary flash evaporator to form a thin film which was then reconstituted by the addition of phosphate buffer of pH 7.4. In case of ether injection method, distilled was heated and a mixture of the lipid dissolved in a small quantity of ether was taken in a syringe and added drop wise to distilled water. In both the cases, the suspension of lipid vesicles so obtained was in the micron size. The suspension sonicated in order to convert the vesicles to nano size. Further they were investigated for their particle size and in-vitro release profile.

Results : It was found that the vesicles obtained after the thin film hydration technique and ether injection method were in the range of 9-11μm and 15-20 μm respectively, after sonication their particle size was in the range of 210-260nm and 290-300nm respectively. They both showed good polydispersity index. It was observed significantly that the nano lipid vesicles that were prepared by thin film hydration technique showed a sustained release of the drug camptothecin, when compared to those prepared by the ether injection method.

Conclusion : Hence it was concluded that the nano lipid vesicles obtained by thin film evaporation technique would retain in the synovial fluid for a longer time due their decreased particle size. Further these nano lipid vesicles would be analysed for their in- vivo effectiveness.


  P129 Category: Rheumatoid Arthritis Top


Lean mass and disease activity are the best predictors of bone mineral loss in premenopausal women with rheumatoid arthritis

Meha Sharma1 , Urmila Dhakad1 , Anupam Wakhlu1 , Danveer Bhadu1 , Deep Dutta2 , Siddharth K Das1 ; Department of Rheumatology, King George's Medical University (KGMU), Lucknow, India 2Department of Endocrinology, Post Graduate Institute of Medical Education & Research (PGIMER) & Dr. Ram Manohar Lohia (RML) Hospital, New Delhi, India

Background: Factors determining bone mineral (BM) loss in young premenopausal women with rheumatoid arthritis (RA) is not known. This study aimed to quantify burden and determine clinical, biochemical, and radiological predictors of BM loss in them.

Methods:
96 female RA patients and 90 age, sex and BMI matched controls underwent clinical and biochemical evaluation, bone mineral density (BMD) and body composition assessment using dual energy X-ray absorptiometry. RA disease activity was assessed using disease activity score 28 (DAS28) and hand X-ray (HXR). Based on a Korean study (low BMD in 91% RA), 88 patients were needed for 80% power and type-I error 5%.

Results:
In young premenopausal females with RA having median symptom and treatment duration of 30 [18-60] and 4 [2-12] months respectively, with moderate disease activity (DAS28 4.88±1.17), occurrence of osteoporosis and osteopenia was 7.29% and 25% at spine, 6.25% and 32.29% at hip and, 17.7% and 56.25% at wrist respectively(significantly higher than controls). Patients with RA had lower BMD at total femur, spine, radius total (RT), and radius ultra-distal. Total lean mass (LM) and BM content was significantly lower in RA (P: 0.022 and <0.001 respectively) (Table-1). In RA, BMD at majority of sites (spine, NOF, GT, RT and radius 33%) had strongest positive correlation with LM followed by body fat percent. RA patients with most severe disease (as per DAS28 & HXR) had lowest BMD at different sites and lowest LM. Stepwise linear regression revealed LM followed by DAS28 score to be best predictors of BMD (Table-2). RA patients receiving oral or intra-articular glucocorticoids did not have significantly different BMD from patients not taking glucocorticoids.

Conclusion:
BM loss is a significant problem in young Indian premenopausal women with recent onset RA with LM and disease severity being the 2 best predictors of BMD.




  P130 Category: Rheumatoid Arthritis Top


Clinical profile and outcome of late-onset rheumatoid arthritis

Ashok Kumar, Mohit Goyal, Mehul Lapsiwala, Anshul Goel, Rahul Bansal; Fortis Flt. Lt. Rajan Dhall Hospital, New Delhi

Background: Onset after the age of 60 years defines late-onset rheumatoid arthritis (LORA). We evaluated the clinical profile and outcome of LORA.

Methods: Seventyconsecutive patients with LORA (ACR-EULAR criteria for RA) were recruited from rheumatology clinic records. RF, anti-CCP, ESR, CRP, tender and swollen joint counts, DAS28-3 (ESR), Indian HAQ, extraarticular disease, comorbidities and treatment details were retrieved. DAS28 scores were obtainedalso at 6 months, 2 years and 5 years. A previous cohort of 90 patients with young onset RA (YORA) from our clinic was used for comparison.

Results: LORA group had a female to male ratio of 2:1. RF and anti-CCP were positive in 52.2% and 50.8% patients (both negative in 40.7%). Mean DAS28 scores at baseline, 6 months, 2 years and 5 years were 5.64, 3.49, 3.45 and 3.36 respectively. Mean HAQ-DI at baseline and 5 years were 1.17 and 1.34.ILD, rheumatoid nodules and Sjogren's syndrome were seen in 4.3%, 7.1% and 4.3% patients respectively. No underlying malignancy was detected in any patient. Conventional DMARDs were the mainstay of treatment and were generally well tolerated. Biologics were offered to 5 but accepted by 2 patients, who then had significant reductions in disease activity. Mean age of onset in YORA was 40.4 years versus 68.2 years in LORA. RF and anti-CCP were positive in 77.9% and 77.5% YORA patients. Mean DAS28 score and tender and swollen joint counts were significantly higher in LORA (p<0.001). Osteoporosis, osteoarthritis, diabetes, hypertension, dyslipidaemia, coronary artery disease and renal disease were significantly more prevalent in LORA compared to YORA. There were no significant differences in adverse events with DMARDs in the two groups.

Conclusion: LORA was frequently a seronegativedisease and yet with significantly higher disease activity. It was accompanied by a high burden of comorbidities and progressive disability.


  P131 Category: Rheumatoid Arthritis Top


Immunocompetent cells may not indicate disease status in rheumatoid arthritis patients

Deepak CL1 , Chandrashekara S1 , Swetha J1 , Renuka Panchagnula2 , Anupama KR1 , Shuchismita Dey1 ;1 ChanRe Rheumatology & Immunology Center & Research, Bengaluru, India,2 ChanRe Diagnostic Laboratory, Bengaluru, India

Background/Purpose: The differential involvement of various immunocompetent cells in the pathogenesis of rheumatoid arthritis (RA) has been well established. The preliminary study investigated the differences in the counts/ratios of immunocompetent cells namely CD3+, CD4+, FoxP3+ and CD19+ in RA patients who achieved remission.

Methods: Seventy-two RA patients who fulfilled the ACR/EULAR 2010 classification criteria were recruited for the study. Fifty-two patients were in remission (DAS28CRP ≤2.6), among whom17 were in deep remission(≤1.6). As per ACR Boolean criteria, 44 patients had remission and 28 had active disease. Twenty patients were DMARD-naïve and had active disease (DAS28CRP >2.6).The demographic and clinical data of all the subjects were recorded, and total counts of CD3+, CD4+, FoxP3+ and CD19+ were analyzed by flow cytometry using serum samples. FoxP3+/CD4+ and CD3+/CD19+ ratios, and percentage of neutrophil to lymphocyte ratio (NLR) were calculated. Group differences for clinicopathological variables were determined separately between active and remission patients classified based on DAS28CRP and ACR Boolean criteria. Correlation among the variables was also examined. Results: The study involved 11 male and 61 female RA patients with a mean age (SD) of 46.54 (10.6) years. The median(range) duration of illness was 45 (3-240) months. The duration of illness and NLRwere significantly different between patients in remission and active disease upon classification based on DAS28CRP criteria. Other demographic/clinical variables such as age, gender, CD3+, CD4+, FoxP3+, CD19+, FoxP3+/CD4+ and CD3+/CD19+ did not differ significantly between the two groups. Duration of illness was moderately negatively correlated and NLR was moderately positively correlated with increase in DASCRP values. Patients classified according ACR Boolean criteria showed group differences only for duration of illness and it was moderately negatively correlated.

Conclusion : CD3+, CD4+, FoxP3+, CD19+ total counts and their ratios did not differ between patients in remission and those with active disease. Hence, they may not indicate the disease status in RA patients classified according to DAS28CRP or ACR Boolean criteria.

Keywords: CD3+, CD4+, FoxP3+, CD19+, RA, DAS28CRP, ACR Boolean criteria


  P132 Category: Rheumatoid Arthritis Top


Study of Insulin Resistance in Rheumatoid arthritis - Patients and its correlation with Subclinical Atherosclerosis

Shafeeque Rahman1 , R.S Taneja2 , Shibani Mehra, Vivek Arya1 ;1 Department of medicine,2 Department of Radiology, PGIMER Ram Manohar Lohia Hospital New Delhi

Background/Purpose: Rheumatoid arthritis (RA) is associated with increased cardiovascular mortality. Traditional risk factors alone do not fully explain the excess cardiovascular risk in RA. Insulin resistance (IR) is known to be increased in patients with established RA and has been shown to be a risk factor for cardiovascular mortality.This study was planned to find the occurrence of insulin resistance in rheumatoid arthritis and its correlation with subclinical atherosclerosis.

Methods: Study included 100 patients diagnosed as RA by ACR/EULAR criteria 2010. Insulin resistance was determined by HOMA2 calculator by fasting blood glucose and fasting insulin. HOMA2IR value > 1 is considered as insulin resistant and those patients were subjected to carotid Doppler to look for carotid intima media thickness(CIMT).Average CIMT (A-CIMT) determined from total 6 readings from both sides.

Results: Out of 100 patients majority were females (83 %), most of patients were in age group 40 -50. Out of 100, 35 % patients had HOMA2 IR value >1. All other parameters are then compared between these two groups, that is IR (HOMA2IR > 1) and Non IR (HOMA2IR ≤1). Factors like age, long duration of disease, high disease activity and RF factor found to be significantly more in insulin resistant group. There is a positive correlation between HOMA2 IR and A-CIMT (r = 0.765, p-value < 0.001) using Pearson's correlation coefficient. ROC curves are made to find critical values of HOMA2IR to predict increased CIMT. The area under the ROC curve is 99.4% (p-value <0.001) which was extremely good.

Conclusion: There is definite occurrence of insulin resistance in rheumatoid arthritis patients. Insulin resistance is correlated with carotid intima media thickness which is a surrogate marker for atherosclerosis


  P133 Category: Rheumatoid Arthritis Top


Comparison Of Disease Activity Score (Das28) And Clinical Disease Activity Index (Cdai) In Patients With Rheumatoid Arthritis

Tushar Gupta, Vivek Arya, R S Taneja; Department of Medicine, PGIMER Ram ManoharLohia Hospital New Delhi

Background/Purpose: The estimated prevalence of Rheumatoid arthritis (RA) in Indian community is 0.75%. Its targeted treatment is based on the assessment of the disease activity. The 'gold standard' to assess disease activity is Disease Activity Score (DAS28). Another index which can be more beneficial to assess disease activity is Clinical Disease Activity Index (CDAI) as it does not require acute-phase reactants and does not involve square-roots and logarithms. Thus, this study was done to compare DAS28 and CDAI to assess the disease activity in RA.

Methods: Cross sectional study conducted including 60 patients diagnosed as RA by ACR/EULAR 2010 criteria above 18 years of age. Each patient was assessed by DAS28 and CDAI index. Multidimensional health assessment questionnaire (MDHAQ) completed by each patient. Statistical analysis was performed to compare disease activity scores of DAS28 and CDAI by using STATA version SE12.

Result: Mean age of patients was 46.05±11.9 years, with mean duration of symptoms 5.8 years.Mean DAS28 was 5.35+1.22, and mean CDAI was 26.47+11.48. Both DAS28 and CDAI showed a strong linear correlation with HAQ. Univariate and multivariate linear regression analysis using HAQ as a dependent outcome measureshowed that DAS 28 and CDAI were significant predictors.Pearson correlation was done between DAS28 and CDAI, which showed significant correlation with coefficient (r) = 0.8919 (p< 0.001). Intra class correlation coefficient measured for DAS28 and CDAI was found to be 0.851 (>0.8), showed perfect intra class agreement between DAS28 and CDAI.

Conclusion: CDAI omits the assessment of acute phase reactants, has strong correlation with DAS28 and is easy to calculate. Thus, CDAI can be used as an alternative to study the disease activity and in planning the treatment.


  P134 Category: Rheumatoid Arthritis Top


Factors influencing the learning capabilities of Methotrexate self- injection techniques among Rheumatic Patients

Rawat R1 , Baghel S2 , Thakran R2 , Messy C3 , Kapoor S5 , Garg SR5 , Malaviya AN4 ; Indian Spinal Injuries Centre, Vasant Kunj,New Delhi,110070

Background/Purpose:
Methotrexate (MTX) is recognised as the gold standard for treatment of rheumatoid arthritis and other inflammatory conditions. The administration of methotrexate using subcutaneous routes increases its therapeutic efficacy, ensure the maximum bioavailability and reduce symptomatic side effects, therefore this route is preferred over oral. However injection form of the drug is less voluntary accepted by the patients due to several reasons. There are numerous studies on the acceptability of MTX self-injections but no major work has been done on the factors that decides its acceptance. This study tried to find out the factor(s) that directly influence the learning capabilities of injection technique.

Methods-(Please avoid any identifiers like Institution Names):
A retrospective data on teaching self-administration of methotrexate were analysed. The collected data included basic information about patients such as age, gender, qualification, occupation and residence (urban or rural area) as well as other details related to disease e.g., duration, diagnosis and family support or willingness to learn injection technique.

Results:
Total 100 patients were registered and divided into two groups, group 1 include 57 patients who learnt self-injection administration technique whereas remaining 43 patients comprised group 2 who unable to learn. No variable proved to be statistical significant except family support with p value 0.0001. Further analysis done on group 1, that revealed late (>2yrs), non-working and rheumatoid arthritis patients preferred to learn self-injection techniques than others.

Conclusion: Among all the variable only family support showed a remarkable association with learning capabilities of injections technique, which is statistical significant also (p =0.0001).


  P135 Category: Rheumatoid Arthritis Top


Safety and Efficacy of biosimilar Rituximab in a cohort of Indian patients with seropositive rheumatoid arthritis

Hegde Arun, Krishnan Shanmuganandan, Vasdev Vivek, Abhishek Kumar, Kartik S, Arjun MN, Kavita S; Army Hospital Research & Referral, New Delhi

Purpose: To study the safety and clinical efficacy of biosimilar Rituximab in patients with seropositive RA, to correlate the disease activity as assessed clinically by the DAS 28 score with levels of various biomarkers like Rheumatoid factor (RF), Anti citrullinated protein antibody (ACPA), and also to correlate the DAS 28 score with functional disease activity, as assessed by the Health Assessment Questionnaire-Disability Index[HAQ-DI],Indian version.

Methodology:
36 adults with moderate disease activity (DAS 28 ≥3.2),who had failed conventional therapy with atleast 2 non biological DMARDs were initiated on Inj Rituximab,1000 mg,given on day 0 and day 15,followed by repeat dosing at 24 wks and 26 wks, after taking informed consent. Serial biomarkers including ESR, CRP, RF and ACPA were estimated at various time points as per protocol. DAS 28 scores and HAQ-DI were estimated at each visit.

Results:
There were statistically significant decline in DAS 28 scores at 24 weeks (p<0.001).75% pts showed a EULAR moderate response whereas 25% showed no response. RF and ACPA titres declined significantly by 69% & 22% from baseline levels respectively. Mean HAQ-DI values declined by 23.5% and 37.1% after the the first and second doses of rituximab. There was a strong positive correlation between HAQ and DAS28[r=0.8, p<0.001].Regression analysis failed to show any significant correlations between ΔDAS and baseline levels of various biomarkers. The most common non infectious adverse effects were infusion reactions (16.6% 0f the pts), mainly seen during the first infusion. There were no serious infections.

Conclusion:
Rituximab is a very safe and efficacious drug for the management of seropositive RA that is refractory to conventional DMARDs. Incidence of infusion related reactions with rituximab are very low, if a proper premedication protocol is adhered to.


  P136 Category: Rheumatoid Arthritis Top


100mg Rituximab enough in RA ?

Dr.Nibha Jain1 ,Dr.Sapan Pandya2 Dr. Puja Srivastava2 ; Senior Resident, Medicine and Rheumatology Dept., V.S. General Hospital, Ahmedabad

Background/Purpose: The approved doses for rituximab in rheumatoid arthritis are 1000mg and 500mg repeated at 2 weeks. Low dose rituximab (100mg) has been variously used in other autoimmune diseases like ITP and also in RA. Since cost of treatment is an issue in our country, we decided to study if 100mg rituximab led to optiumum B cell depletion and also clinical efficacy in our patients with DMARD refractory RA. Our aim was to study the efficacy and safety of low dose rituximab.

Methods: Each patient received 100mg of rituximab 1 week apart for 4 weeks. Only seropositive patients refractory to DMARDs and biologic naïve were included. Baseline activity scores DAS28, CDAI, SDAI, RAPID and HAQ were calculated along with CD19 levels as a marker of B cell depletion. Patients were re-evaluated at week 6 while CD19 levels were repeated at week 1 and 6.

Results: Results show 5 out of 7 patients had clinical improvement which was reflected by change in DAS28 of >1.2. 'p' values were significant for DAS28, HAQ, RAPID and CDAI at 6 weeks when compared to baseline. CD19 levels decreased to 0 from baseline in all the patients and remained 0 till week 6. No major adverse effects were noted.



Conclusion: Low dose rituximab (100mg) seems effective in patients with DMARD refractory RA with significant improvement seen at 6 weeks. B cells are fully depleted as early as week 1 in most seropositive patients. We are further following up these patients for evaluation at 3 and 6 months both in terms of maintenance of clinical response and B cell repopulation.


  P137 Category: Rheumatoid Arthritis Top


Study of Rheumatoid Arthritis Patients In A Tertiary Clinic

Dr. Nibha Jain1 , Dr. Rutviz Mistry1, Dr. Puja Srivastava2 , Dr. Sapan Pandya2 ; V.S. General Hospital, Ahmedabad

Background/Purpose: In a resourcepoor setting as our where patients cannot afford CRP values, clinical indices would be easier to apply for monitoring disease activity in RA. We studied the correlation between DAS 28 (ESR), CDAI and RAPID in our patients of RA. We also looked at proportion of patients achieving remission and or low disease activity at first follow up and the baseline features that led to it.

Methods: Observational retrospective study at Government hospital and medical college from October 2015 to August 2016. All patients fulfilling ACR 2010 criteria for RA were included. All clinical and laboratory parameters of relevance were analysed by descriptive statistics. DAS 28 (ESR) was correlated with CDAI and RAPID scores for patients where all data was available. DAS 28 responses were calculated at first follow up and compared to the baseline features of those in remission/low disease activity from those who did not achieve the same.

Results: Total 251 patients were studied with mean age: 47.14 +11.8. Total duration of illness 1.56 + 5.6 years on presentation. RF was positive in 111 patients out of 125, 59 had ACCP positive out of 63 while 42 were both RF and ACCP positive.



Follow up data could be retrieved from 65 patients at a mean duration of 3.1 months. 40% (26) achieved remission or low disease activity defined as DAS28<3.2. Comparing the baseline variables between those in remission/low disease activity versus those not in this group revealed seropositivity both for CCP and RF and presence of comorbities as being significantly different. DAS 28 (ESR) correlated well with CDAI and RAPID 3 scores.





Conclusion:
The CDAI and RAPID 3 can be substituted in place of DAS 28 scores for assessing disease activity in patients of RA. In our group, 40% achieved EULAR remission and or low disease activity at first follow up and those who were seropositivity for RF and or CCP and absence of any comorbidities were significant contributors to this.


  P138 Category: Rheumatoid Arthritis Top


Comparison of Combination Disease Modifying Antirheumatic Drugs (DMARDs) with Methotrexate Monotherapy in Early Rheumatoid Arthritis: An Open Label Randomized Trial.

Jignesh Usdadiya, Sonal Mehra, Vikramraj Jain, Bharat Singh, Dantis Emmanuel, Ankit Jain, Mithun CB, Durga Prasanna Misra, Vir Singh Negi; Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry

Background/Purpose : Treatment recommendations for patients with active rheumatoid arthritis (RA) with poor response to initial methotrexate (MTX) monotherapy propose a varied therapy. This study was planned to evaluate clinical efficacy and safety of combination DMARD therapy [MTX + leflunomide (Lef) + hydroxychloroquine (HCQ)] versus MTX monotherapy in patients with early RA.

Methods : Patients with DMARD naïve RA (duration < 1 year) were randomized to receive either combination DMARD therapy or MTX monotherapy. The primary outcomes were a proportion of patients who achieved a good response according to European League Against Rheumatism (EULAR) response and functional ability with Indian version of health assessment questionnaire (IHAQ) at the end of 3 months. In intention to treat analysis, we used non-responder imputation.

Results : Seventy-four patients were randomized to each therapy arm. At 12 weeks, 42 (58%) patients in combination arm achieved a good EULAR response compared with 22 (30%) in monotherapy arm (p<0.001). The median IHAQ was 0.33 in combination group compared with 0.91 in monotherapy group (p=0.001). The median change in IHAQ was -1.25 in combination group and -0.92 in monotherapy group, p=0.005. Patients treated with combination therapy had better outcome for other variables than those treated with MTX: a lower mean disease activity score in 28 joints (DAS28) (3.29 vs. 4.03, p<0.001); a higher mean fall in the DAS28 score (-2.41 vs -1.58, p<0.001); and a higher proportion of patients with low disease activity state (DAS28≤3.2) (55% vs 30%, p=0.004). There was a significant reduction of synovitis sum scores in grey-scale ultrasound (GSUS) and power Doppler ultrasound (PDUS) in patients treated with combination therapy. Adverse events were similar between both therapy arms.

Conclusion : Combination of MTX plus Lef plus HCQ, a less well-studied combination therapy in RA, is better than MTX monotherapy in early RA with similar safety profile.


  P139 Category: Rheumatoid Arthritis Top


Clinical evaluation of dry eyes in patients with Rheumatoid Arthritis

Shiva Prasad B.N, Phanindhara Reddy Y1 ; Consultant Rheumatologist and Clinical Immunologist, Apollo BGS Hospital, Mysore,1 Mysore Race Club Eye Hospital, Mysuru, Karnataka

Background : To detect dry eye and to assess the relation with Rheumatoid Factorand severity of disease in Rheumatoid arthritis.

Materials and Methods
: A cross-sectional study was done from December 2013 to October 2015. Fifty nine Eyes of 30 patients diagnosed as Rheumatoid arthritis age > 18 years, with disease duration more than five years were included. Rheumatoid Factor positivity was noted and severity of disease was calculated with DAS28-ESR. Dryness of eye wasassessed by Tear Film Breakup Time (TBUT),Fluoresceinstaining and Schirmer's Test I.

Results: Out of 120 patients assessed, 30 patients were included in the study. Out of 59 eyes 42.4% had TBUT <10seconds(mean 6.96 ± 1.54 seconds), 37.3% had Schirmer's I ≤ 5mm at 5 minutes (mean 3.4 ± 1.05 mm) and fluorescein staining was positive in 33.9%. 42.4% were diagnosed with dry eye.Out of 51 eyes with positiveRheumatoid factor 24 haddry eye but 1 out of 8 eyes with negative Rheumatoid factor had dry eye(p=0.066).Meanseverity score of diseasebased on DAS28-ESR was 2.9 in dry eyeand 4.1 in patients withoutdry eye.

Conclusion: Dry eye in Rheumatoid Arthritis can be detected by simple tests. There is no significant association between Rheumatoid factor, disease severity and dry eye.


  P140 Category: Rheumatoid Arthritis Top


Abatacept in the management of Refractory Rheumatoid Arthritis: Experience at a teritiary care centre

Arjun MN, ArunHegde, VivekVasdev, GD Choudhury, Kunal Kishore, Abhishek Kumar; Department of Rheumatology & Clinical Immunology, Army Hospital Research & Referral, Delhi Cantt

Background: T cells are pathogenic in rheumatoid arthritis (RA) and have an important role in sustaining synovitis even in established disease. Modulation of T-cell activity by blocking of costimulatory signals suppresses inflammation and improves prognosis in RA. Aim of this study is to study the effect of Abatacept in patients suffering from refractory rheumatoid arthritis with high disease activity and inadequate response to DMARDs

Methods:
A total of 60 patients of established RA attending the rheumatology clinic between Oct 2013- Jul 2016, who had been refractory to at least 2 non biological DMARDs including methotrexate, were included in the study. All the patients had moderate disease activity as measured by the DAS 28- ESR score (>3.2) and CDAI (>10). All patients received monthly infusion of Injection Abatacept500 mg for 12 months. Patients were reassessed for disease activity after 6 months and 12 months.

Results:
Out of 60 patients, 48 were female and 12 were male. 5 patients had history of pulmonary tuberculosis in the past, and had received anti tubercular treatment. Mean age of patients was 42.20 ± 12.08 yr. Mean disease duration was 7.86 ± 2.6 yrs. Mean DAS 28 at baseline was 5.9 ± 0.6 whereas mean CDAI at baseline was 26.8 ± 3.2.There was significant improvement in mean DAS 28 score [DAS 28: 3.7 ± 0.48, and 2.6 ± 0.86 at 6 and 12 months (p <0.0001) respectively], and mean CDAI scores [CDAI: 9.0 ± 3.48, and 3.2 ± 1.4 at 6 months and 12 months (p <0.001) respectively], as compared to baseline. There were no adverse effects in form of infusion related reactions in any of the patients. There was no reactivation of tuberculosis

Conclusion : Abatacept is an effective and generally well tolerated treatment option for RA patients with an inadequate response to methotrexate.


  P141 Category: Rheumatoid Arthritis Top


Severity of Interstitial lung disease and seropositivity in patients with rheumatoid arthritis.

Sowndhariya V Annamalai, Rajeswari Sankaralingam, Tamilselvam Tiruchengode Natesan, Balameena Selvakumar, Saravanan Mayilsamy, Sivakumar Vengudusamy, Vignesh Mantharam, Mythili Seetharaman, Balaji Chilukuri, Ramesh R; Institute of Rheumatology, Madras Medical College, Chennai

Background: Interstitial lung disease (ILD) is the second leading cause of mortality in patients with rheumatoid arthritis. Association of RF and ACPA positivity with the presence of interstitial lung disease is already well established.

Objectives:
To find the association between rheumatoid factor (RF) and anti cyclic citrullinated peptides (ACPA) positivity with severity of interstitial lung disease(ILD) in patients with rheumatoid arthritis.

Results:
A cross sectional study was conducted at our centre by reviewing the data of 483 patients with rheumatoid arthritis from July 2013- July 2016. Of these patients, 26 patients who had interstitial lung disease were taken and their clinical characteristics, laboratory features, type and severity of ILD were analysed. Mean age of patients was 49.5±11 years. 85% were females and 15% males; 84.6% (22/26) of patients had usual interstitial pneumonia (UIP), 15.4% (4/26) of patients had non specific interstitial pneumonia (NSIP). None had organising pneumonia or lymphocytic interstitial pneumonia. 50% (13/26) of patients had RF positivity whereas 61.5%(16/26) were ACPA positive. 30.7% patients were negative for both antibodies. Patients with high titres of ACPA(>200 RU/ml) had higher scores in the computed tomography semi quantitative scoring of ILD (p value=0.03),though not associated with severity of forced vital capacity. There was no significant association between presence of rheumatoid factor (p=1.00) or anti cyclic citrullinated peptide antibodies (p=0.6) with NSIP or UIP pattern of interstitial lung disease.

Conclusion:
High titres of ACPA have significant association with increased severity of interstitial lung disease as assessed by semi-quantitative CT scoring. Presence of RF and anti cyclic citrullinated peptide antibodies does not have any significant association with any specific pattern of interstitial lung disease in rheumatoid arthritis.


  P142 Category: Rheumatoid Arthritis Top


EPCs and pro-inflammatory cytokines in seropositive Rheumatoid Arthritis

Ashit Syngle1 , Nidhi Garg2 , Pawan Krishan3 ; Cardio Rheuma & Healing Touch City Clinic, Chandigarh & Consultant Rheumatologist- Fortis Multi Speciality Hospital, Mohali; Department of Pharmaceutical Sciences & Drug Research, Punjabi University; Department of Pharmaceutical Sciences & Drug Research, Punjabi University, Patiala, Punjab

Purpose: We aimed to investigate the relationship between Endothelial Progenitor Cells (EPCs) and pro-inflammatory cytokines in seropositive and seronegative Rheumatoid Arthritis (RA).

Methods: Forty adult RA patients were enrolled in the study and divided into seropositive (n = 20) and seronegative (n = 20) groups. EPCs (CD34+/CD133+) were quantified by flow cytometry and pro-inflammatory cytokines (TNF- α, IL-6 and IL-1) was measured by using standard ELISA kits. As surrogate for disease activity, DAS28, C-reactive protein and ESR levels were determined.

Results:
EPCs were significantly decreased in seropositive patients as compared to seronegative patients (0.021 ± 0.001% versus 0.034±0.001%, P = 0.01) in RA. Levels of pro-inflammatory cytokines i.e. TNF- α, IL-6, and IL-1 were significantly (P = 0.01) higher in seropositive patients as compared to seronegative. Significant negative correlation was observed between the number of EPCs and IL-1, TNF-α and disease activity.

Conclusion: Seropositivity plays a major role in EPC biology which is characterized by reduced peripheral concentration of EPCs and higher concentration of pro-inflammatory cytokines in seropositive patients as compared to seronegative RA patients. Higher cardiovascular risk in seropositive patients is possibly also contributed by reduced EPCs and higher level of proinflammatory cytokines.


  P143 Category: Rheumatoid Arthritis Top


The analysis of Alfa_enolase in serum & synovial fluid of patients with Rheumatoid Arthritis

Saghiri R1 , Ebrahimi_Rad M1 , Akhbari H2 , Valadbage S1 , Karami f1 ;1 Department of biochemistry Pasteur institute of iran IRAN, Medical University of Birjand,2 Department Rheumatology

Background and Objective : One of the most common systemic autoimmune is rheumatoid arthritis, (RA). It is about 1% of world population that are affected. Patients with RA often certain antibodies against proteins containing citruline produced. One of the proteins citrullinated alpha enolase that' s synovial membrane is seen in people with arthritis. Role alpha enolase in patients with rheumatoid arthritis is very important.

Material and Methods : In this study, serum samples from 20 healthy subjects and 40 samples of serum and 20 samples of synovial fluid of patients with rheumatoid arthritis sampling method based on criteria American College of Rheumatology (ACR) diagnostic view of rheumatologist selected. Electrochemical method for measuring the molecular weight of the enzyme as well as to measure the activity of alpha enolase ELISA is used.

Result : Alpha enolase activity in synovial fluid of patients is (44.92±7.0)mg/ml and in healthy is (0.76±0.12)mg/ml. Alpha enolase activity in serum of patients is (4.49±1.34)mg/ml and in healthy is (0.76±0.53)mg/ml.Which increases the activity of the patients was significant statistically (p<0.001).

Conclusion : According to the results of this study range of alpha enolase activity in patient with Rheumatoid Arthiritis compared to the healthy population increased.


  P144 Category: Rheumatoid Arthritis Top


Prescribing patterns, compliance and response at first follow up in RA patients: a multicenter study

Sapan Pandyaψ , Pradip Prajapati, Alpana Parmar, Reena Sharma, Namisha Patel, Puja Srivastava, Shabbir Chikani, Taral Parikh; Vedanta institute of medical sciences, Ahmedabad

Background/Purpose : Compliance to DMARDs is always a concern especially with combinations being used upfront, more so in the Indian context due to socio-economic reasons. We studied the compliance of patients of RA at the first follow up for the prescribed drugs and reasons for non compliance. We also looked at the prescribing patterns and DAS 28 responses at the first follow up from different centers of Gujarat

Methods : Ours was a prospective observational study of patients fulfilling ACR 2010 criteria for RA from 8 centers of Gujarat. The study duration was from June to Sept'2016..Baseline data was recorded in paper proformaswhich included DAS 28, CDAI scores and prescribing patterns. First follow up data was recorded as - interval for first follow up, DAS 28, CDAI, HAQ. For each drug, reasons for non compliancewere noted. Baseline descriptive data and DAS 28 responses at first follow up were analysed.

Results:



Conclusions: At a mean first follow up of 8 weeks, more than three fourths of the patients were compliant to most DMARDs. Most non compliance was not due to drug side effects. Rheumatologists prescribed the combination of MTX and HCQ in more than 80% of patients. At first follow up, about a fourth of patients achieved DAS 28 remission or low disease activity while half had a good EULAR response. The study is being continued for longer follow up data


  P145 Category: Rheumatoid Arthritis Top


Bone Mineral Density in Indian Female Patients with Rheumatoid Arthritis

Urmila Dhakad, Rasmi Ranjan Sahoo, Meha Sharma, Danveer Bhadoo, Durgesh Srivastav, Saumya Ranjan Tripathy, Hari Krishnan V, Siddharth Kumar Das; Department of Rheumatology, King George Medical University, Lucknow-226003, India

Background/Purpose: Low bone mineral density (BMD) is prevalent among rheumatoid arthritis (RA) patients. It has been hypothesized that bone loss in RA may be reflective of the disease activity. However, its association with disease activity is not reported consistently. Pattern of BMD and its relation with disease activity among Indian RA patients has not been evaluated. This study aims to show the pattern of low BMD and its relation with disease activity among premenopausal RA patients in India.

Methods: 115 confirmed RA (fulfilling ACR/EULAR criteria) premenopausal female patients (age range; 18-45 years) along with 90 age and sex matched healthy controls were recruited prospectively. Routine hematological tests, vitamin D level and Dual Energy X-ray Absorptiometry (for estimation of BMD at proximal femur, lumbar spine (L1-L4), and distal radius) were done in all patients. In RA patients, Rheumatoid factor (RF), anti citrullinated protein antibody (ACPA) and Clinical disease activity index (CDAI) were also assessed.

Results: Mean age of patients and control were 33.2+6.8 and 37.7+5.03 years, respectively. Mean BMI of patients and controls were 21.9+4.6 and 22.95+2.62 respectively. Mean duration of disease among patients was 4.2+3.4 yrs. RF was positive in 79.4% of patients whereas ACPA was positive in 89% of patients. Mean vitamin D level among patients was 30.3+25.01 ng/ml. 83.5% patients were on steroid for less than 3 months. Low BMD was seen in 9.5% of patients at L1-L4 (vs. 3.33% of controls, p: 0.09), 15.6% at radius(vs. none in controls, p<0.0001) and 4.3% at neck of femur(vs. none in controls, p=0.06). BMD at L1-L4, neck of femur and radius did not correlate with swollen joint count, ESR, CRP and CDAI.

Conclusion: Low BMD at radius is prevalent among premenopausal female RA patients in India. However, it did not correlate with swollen joint count, ESR, CRP and CDAI.


  P146 Category: Rheumatoid Arthritis Top


Eosinophilia in rheumatoid arthritis: A study of the etiology and clinical significance

Dantis Emmanuel, Ankit Jain, Chengappa KG, Gaurav Seth, Sonal Mehra, Jignesh Usdadiya, Vikramraj Jain, Durga Prasanna Misra, and Vir S Negi; JIPMER, Pondicherry

Background/Purpose: Eosinophilia is found in upto 7% of patients with rheumatoid arthritis (RA) and may be due to numerous causes. The aim of the study was to investigate the etiology of eosinophilia in RA, its association with disease characteristics and response to antihelminthic therapy.

Methods: This prospective observational study included patients satisfying ACR/EULAR 2010 criteria for classification of RA who were having persistent eosinophilia. The presence of eosinophilia was defined as an absolute eosinophil count (AEC) above 500/mm3. Eosinophilia was classified as mild (<1500/mm3), moderate (1500-5000/ mm3) or severe (>5000/mm3). Causes of eosinophilia and its response to antihelminthic therapy with Albendazole were studied. Disease activity (DAS28ESR) was assessed in all patients. Patients were followed up for a minimum period of one year.

Results: We studied 147 patients (136 females) with RA. Mean age of patients was 47.8+10.4 years. All patients had mild to moderate eosinophilia except one. Sixty percent (60%) of the patients were either in remission or had low disease activity at the time of inclusion in study. Intestinal helminths were found in 31 patients (21.09%); atopy, allergy, bronchial asthma, eczema and bronchiectasis were found in 26 patients (17.69%); in the remaining 90 patients (61.22%), no obvious etiologies could be found. In eighty-nine patients (60.54%) eosinophilia disappeared after antihelminthic therapy. No difference could be found when disease activity was compared between those who had response to Antihelminthic therapy compared to those who had a persistent eosinophilia at the initiation of study and at follow up (p value 0.41 and 0.96 respectively).

Conclusion: Eosinophilia in patients with Rheumatoid arthritis is more likely due to secondary causes especially intestinal parasites and does not seem to be associated with disease activity.

P147 Category: Rheumatoid Arthritis

Predictors of autonomic neuropathy in rheumatoid arthritis


Ashit Syngle1, 2 , Vijaita Syngle1 , Nidhi Garg3 , Pawan Krishan3 , Inderjeet Verma3 ;1 Cardio Rheuma and Healing Touch City Clinic, Chandigarh, India,2 Rheumatologist Fortis Multi Specialty Hospital, Mohali, India,3 Department of Pharmaceutical Sciences and Drug Research, Punjabi University, Patiala, India

Background: Autonomic dysfunction occurs in rheumatoid arthritis (RA). However, the association between the autonomic dysfunction and inflammation has not been investigated in RA. We investigated the relationship between inflammation and ANS function in RA.

Methods: In this cross-sectional study, 25 RA patients and 25 age and sex-matched healthy controls were recruited. Autonomic function assessed by five cardiovascular reflex tests according to Ewing. Parasympathetic dysfunction established by applying three tests: heart rate response to deep breath (HRD) and standing (HRS) and Valsalva tests. Sympathetic dysfunction examined by applying two tests: BP response to standing and handgrip test. Peripheral sympathetic autonomic function assessed by Sudoscan through measurement of electrochemical skin conductance of hands and feet. Sudoscan investigates the sweat gland activity and used as a surrogate to study the damage of sympathetic sudomotor nerves in neuropathy. It is an indirect assessment tool of sudomotor function. Disease-specific and inflammatory measures (DAS 28, ESR, CRP, TNF-α, IL-6 and IL-1) were determined.

Results: RA patients had significantly impaired HRD, HRS, BP response to hand grip and sudomotor function as compared to healthy controls. Pro-inflammatory cytokines were significantly higher in RA as compared to healthy controls (p < 0.05). DAS 28 significantly correlated with HRD in RA. ESR significantly correlated with HRD and HRS. TNF-α significantly correlated with HRD, HRS, BP response to standing and sudomotor function. Significant correlation was found between IL-6 and HRS. Seropositive patients had more pronounced CAN and sudomotor dysfunction.

Conclusion: Autonomic dysfunction in RA is related to disease activity, seropositivity and pro-inflammatory cytokines.


  P148 Category: Rheumatoid Arthritis Top


Patterns Of Thyroid Dysfunction In Patients With Rheumatoid Arthritis And Its Association With Disease Activity And Duration

Mahendra Debbarma, Prasanta Dihingia, Sreemanta Madhab Baruah, Tridip Kumar Das, Sanjeev Kakati; Department of General Medicine, Assam Medical College and Hospital

Background/Purpose: Cardiovascular complications is one of the comorbid conditions in rheumatoid arthritis (RA). This study was done to determine the pattern of thyroid dysfunctions in Rheumatoid Arthritis patients as hypothyroidism increase the cardiovascular risk.

Materials and Methods: A hospital based case-control study was done on 103 patients based on the Classification Criteria for Rheumatoid Arthritis (ACR/EULAR2010)and 103 healthy volunteers were taken as the control group. Thyroid function test was done on cases and controls and the patterns of different thyroid dysfunctions were compared in between the two groups.

Results: Thyroid dysfunction was present in 22(21.35%) patients, 12 patients had subclinical hypothyroidism and 6 had hypothyroidism, 4 patients had subclinical hyperthyroidism whereas in the control group 5 (4.85%) had thyroid dysfunction. Majority of the thyroid dysfunction patients(13) were in the moderate disease activity group and 11 of the 22 patients were associated with Anti-CCP+ and RF+.

Conclusion: The study shows that thyroid dysfunction was found in significant number of patients with Rheumatoid Arthritis compared to the control group. Therefore, advising these tests in RA patients is recommended and thereby correction of comorbid conditions with early diagnosis can halt an aggravating RA course.


  P149 Category: Rheumatoid Arthritis Top


Glucocorticoid-sparing in patients suffering from rheumatoid arthritis and treated with tocilizumab

Joseph T Antony, Jayaraj K, Paul T Antony, Geo Paul, T P Antony; Departments of General medicine, Clinical Immunology, Amala institute of medical sciences, Thrissur

Objectives: To describe steroid-sparing in rheumatoid arthritis (RA) patients treated with tocilizumab (TCZ).

Methods: To evaluate the proportion of RA patients treated with more than 5 mg of prednisone (or equivalent)/day and starting TCZ who can reduce their steroid dose to less than 5 mg/day after 3 months without intensification of disease-modifying anti-rheumatic drugs (DMARDs). We included patients with moderate-to-severe RA, >18 years old, starting TCZ and receiving corticosteroids (GCs) at a dose greater than 5 mg/day of prednisone for at least 3 months.

Results: Amongst the 15 analysed patients (78% women, median RA duration: 8 years, mean DAS28-ESR: 5.1±1.3), 65% were able to discontinue steroids by third month. Further 25% were able to reduce their steroid doses all without conventional synthetic (cs) DMARD intensification. Predictive factors were RA duration of 5 years or less, daily prednisone dose of 7.5 mg or less, and high ESR value before the first TCZ infusion. Disease activity improved over the 1-year period (DAS28-ESR LDA and remission in81 % and 70% of patients at M6, respectively). Amongst the 15 patients analysed for safety, at least one AE and at least one SAE were reported in 10 patients(67%) and in 2 patients (15%), respectively. No unexplained safety signal arose with TCZ.

Conclusions: A biological DMARD as TCZ allows reducing both GCs dose and disease activity in RA patients.


  P150 Category: Rheumatoid Arthritis Top


Outcome Of Rheumatoid Arthritis Patients: A Retrospective Study

Anuj Singhal, Rahul Tyagi, Vivek Hande, SK Joshi; Department of Rheumatology, Pulmonolgy, General medicine and Cardiology

Background and Objective: Rheumatoid arthritis (RA) is a major burden in India. Factors determining remission have been studied in Western population but not in India. Hence, the objective was to find out the factors determining low disease activity (DAS28 < 3.5) and the efficacy of biologics alone or as add on in RA patients.

Materials and Methods: This was a single centre, tertiary care hospital study carried out in South Mumbai, India. Retrospective database was collected between January 2014- June 2016 from hospital medical records department, lab records department, OPD register and pharmacy records. Patients with RA as per ACR criteria were enrolled into the study. DMARD failure patients were put on methotrexate (MTX) treatment along with single biologic or as add-on biologic. To monitor disease activity, disease activity score 28 was obtained at baseline, and after 3 months and 6 months of therapy initiation. Logistic regression applied for dichotomized outcome variable i.e DAS28<3.5 was analyzed.

Results: A total of 135 RA patients (F=100, M=35) with mean age of 44 years were subjected to either; MTX+ Hydroxychloroquine (HCQ) (n=73) or MTX+ Leflunomide (LEFT) (n=22) or HCQ+LEFT (dual n=2, single n=91) or MTX+ Biological response modifiers (BRM) (n=51) or MTX+ 2 types of BRM (n=23). The mean duration of treatment was 5 months. The RF titre values were positive for 90 patients and negative for 20 patients. The ACPAb titre values were positive for 86 patients and negative for 26 patients. Initial DAS28 was 4.10±1.13 (mean±SD) which reduced to 3.58±0.98 at 3 months of treatment and further reduced to 3.11±0.69 at 6 months of treatment. About 48.1% and 70.4% of patients attained remission at 3 months and 6 months of treatment, respectively.



Patients who have received the biological treatment had statistically significant logistic regression, odds ratio of 2.77(1.0, 7.62, 95% CI) or approximately 3 times more pain relief as compared no biologic treatment. The data on regression of patients who were shifted from one biologic to another biologics was poor due to less sample size for each biologic. Patients who had remission at baseline (DAS28), their chance of pain relief was less, 0.24 compared to who did not have remission, statistically significant.

Conclusion: Higher disease activity at baseline and use of biologics were predictors of better outcomes in terms of DAS28 score at the end of 6 months.


  P151 Category: Rheumatoid Arthritis Top


Nerve conduction study in rheumatoid arthritis patients: a case - control study

Dr Satarupa dash, Dr Atanu K Thakur; Department physiology, Department medicine, VIMSAR, BURLA, Odisha

Background/Purpose: To evaluate the nerve involvement in rheumatoid arthritis patients through the nerve conduction study.

Methods: Thirty normal subjects and rheumatoid arthritis patients for each were included in this study, the normal subjects were matched regarding the age and sex with rheumatoid arthritis patients. Rheumatoid arthritis patients were diagnosed according to American revised criteria (ARC) 1987; at the Department of Medicine in VIMSAR the period of 15th of November 2014 - 15th of May 2015.Patients with Diabetes, Sickle cell disease,thyroid disorder are excluded from study. NCS were done for each patient and normal subject by using the Neurostim NS2 machine.Nerve studied were median, ulnar, radial, posterior tibial and common peroneal.The study includes distal motor and sensory latency (DML, DSL) compound motor and sensory action potentials (CMAP, SNAP) motor and sensory nerve conduction velocities (MNCV, SNCV). Collected data were analysed by (SPSS) software; unpaired t-test was used to compare between the studied parameters. P-value equal to or less than 0.05 is considered to be significant.

Results: In this study, peripheral neuropathy was detected in 18 patients (56%); mononeuritis simplex was the commonest lesion and detected in 12 patients (66.6%) out of the 18 patients. The entrapment neuropathy was found in 8 patients (42.84%), affecting the median 4 (21.47%), posterior tibial 2 (14.81%) and ulnar 1(7.10%) nerves. Mononeuritis multiplex was detected in 4 patients (20.37%), and symmetrical polyneuropathy found in 2 patients (12.90%).

Conclusion: This study demonstrates the involvement of peripheral nerves in rheumatoid arthritis patients, which remain subclinically. As NCS is a non-invasive techniques should be recommended in patients ofRheumatoid arthritis as a routine for early detection of peripheral neuropathy.


  P152 Category: Rheumatoid Arthritis Top


Clinical profile of patients with Rheumatoid Arthritis and Hypothyroidism

Nagnath R khadke, Arvind Chopra; CRD, Pune

Background/purpose: studies have shown increased prevalence of hypothyroidism in patients with rheumatoid arthritis (RA) and it adversely affects the disease activity.

Methods: Single centre retrospective cross sectional study. Study period - may 2013 to march 2016. Data collected from referral database of CRD,pune maintained since 1996 with over 60,000 patients. Diagnosis of RA clinical. 2010 ACR -EULAR criterion applied. Diagnosis of Hypothyroidismbased on prior health records or Thyroid function tests. Clinical profile, lab parameters, Serological tests (RF, ACCP, ANA) recorded. Thyroid function test (TFT) done as needed.RA disease activity indices (DAS 28 ESR, HAQ, pain VAS) and treatment details noted at baseline. The data of comparison with age, gender matched 125 patients of rheumatoid arthritis will be extracted and presented. Statistical analysis done by SSPMS software.

Results: Total 125 patients with first visit in study period identified. Female - 119 (95.2%). Male - 6 (4.8%). Positivity - RF (70.16%), ACCP (81.6 %). 2010 ACR-EULAR criterion satisfied by 87.82 % (n-115). RA characteristics - average age at onset (F/M- 38.22/44.5 yrs), average at presentation (43.17/48.5), average age at onset (4.90/4.12 yrs). Hypothyroidism characteristic - Average age at onset (36.4/44.5 yrs), average disease duration (6.56/6.12). Mean DAS 28 ESR (5.97/5.33), mean ESR (58.82/34.83), mean HAQ (7.43/8.33). NSAIDs, Methotrexate and steroids initiated in 90.9% / 70.4%/ 70.4% patients respectively.

Conclusion: Patients with rheumatoid arthritis should be actively evaluated for the presence of hypothyroidism and it adversely affects the disease activity and outcome.


  P153 Category: Myositis Top


High prevalence of asymptomatic vertebral fractures in inflammatory myositis

Latika Gupta, Sukesh Edavalath, Ramnath Misra, Able Lawrence; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

Background/Purpose: Patients with inflammatory myositis have increased prevalence of symptomatic fractures. Early detection of asymptomatic vertebral fractures may help in their prevention. We studied the frequency of asymptomatic vertebral fracture and its risk factors in inflammatory myositis.

Methods: Adults with inflammatory myositis were included and dorsal and lumbar spine lateral radiographs were taken. The scoring was done using Genant's semi-quantitative method. Besides demographic data, weight, height, postmenopausal status, duration of corticosteroid use, anticonvulsants, calcium supplements, and other co-morbidities like diabetes or hypothyroidism and past history of non-vertebral fracture were recorded. Bone mineral density was done using DEXA. Myositis Damage Index (MDI) was also assessed. All results are expressed in median and IQR.

Results: 100 patients (82 females) with myositis of age 35.5 (28.5-46) years and disease duration 3 (1.81-8.0) years were studied. Twenty patients were postmenopausal women. 35 patients had adult dermatomyositis (DM), 26 each had polymyositis and 26 connective tissue disease associated myositis and 13 had juvenile onset myositis. Forty-six patients had asymptomatic vertebral fractures. 19 patients had more than one fracture. Half the fractures occurred in those with disease <5 years. Of the 69 fractures, 47 (68.1%) were mild, 16 (23.2%) were moderate and 6 (8.7%) were severe. 11th and 12th thoracic vertebrae were most commonly affected. Of the 67 patients who underwent BMD assessment, 62.7% were osteopenic and 26.9% were osteoporotic. T scores of DEXA scan at the lower third of the radius correlated negatively with fracture number (r=-0.27 (-0.50 to -0.005), p=0.04). Gender, age, duration of disease, intake of corticosteroid or calcium, BMI, menopausal status, or MDI had no correlation with number of fractures.

Conclusion: Patients with inflammatory myositis have high prevalance of asymptomatic vertebral fractures.


  P154 Category: Myositis Top


Comparison of clinical profile & treatment in idiopathic inflammatory myositis & mixed connective tissue disease related myositis

Kakade Girish, Balakrishnan C, Sagdeo Parikshit, Shah Romi, Joshi Manohar; P D Hinduja National hospital & Medical Research Centre, Mumbai

Introduction: Mixed connective tissue disease (MCTD) patients commonly have myositis. This study was done to compare clinical profile & treatment in MCTD Myositis & idiopathic inflammatory myositis (IIM).

Methods: In this retrospective study, we collected data from medical record files of patients of IIM & MCTD myositis. We recorded demographic profile, clinical features, treatment, response to treatment, number of flares & adverse events.

Results: In MCTD myositis, we had 11 patients (1 male, 10 females). Mean age at diagnosis was 33.91 yrs. Mean follow up duration was 38.18 months. Mean creatine phosphokinase (CPK) & lactate dehydrogenase (LDH) levels at diagnosis were 3591.14 & 570.01 IU/L respectively. 3 patients achieved remission with single immunosuppressant; while 8 patients required addition of second immunosuppressant. No patient required intravenous immunoglobulin or rituximab therapy. 4 patients had flares in follow up. There were no malignancies in this group. In IIM, we had 21 patients (4 males, 17 females). Of these, 15 had dermatomyositis & 6 had polymyositis. Mean age at diagnosis was 46.90 yrs. Mean follow up after diagnosis was 48.90 months. Mean CPK & LDH levels at diagnosis were 3420.21 & 966.54 IU/L respectively. 5 patients had malignancy either at diagnosis or developed in follow up. Of these, 2 had carcinoma brest, 1 carcinoma tongue, 1 myelodysplastic syndrome & 1 neuroendocrine malignant tumour. Of these 5 patients with malignancy, 4 had dermatomyositis. 5 patients required single immunosuppressants & 11 required 2 immunosuppressants. 4 patients required intravenous immunoglobulin therapy. 1 patient required additional rituximab therapy. 7 patients had flares in follow up.

Conclusion: IIM patients were older at diagnosis & required intravenous immunoglobulin or rituximab therapy more commonly as compared to MCTD associated myositis in our study.


  P155 Category: Myositis Top


Cardiovascular Involvement In Severe Dermatomyositis - A Retrospective Study In Tertiary Care Centre In South India

V A DeepikaPonnuru1 , Varaprasad IR2 , Rajasekhar L3 ; Nizam's Institute of Medical Sciences, Hyderabad

Introduction: Cardiac involvement is most common cause of mortality in Dermatomyositis.

Objective: To study the cardiovascular involvement of patients with Dermatomyositis admitted in a tertiary care centre in South India.

Methods: In this retrospective study, hospital records of patients admitted with dermatomyositis who satisfied Peter and Bohan's criteriabetween January 2012 and August 2016 were reviewed and included. Demographic data, clinical features,laboratory details were noted.CPK>200IU/L, SGOT>3xULN, LDH>400IU/L were taken as abnormal.Cardiac involvement was defined as presence of Pulmonary arterial hypertension(PAH), RVSP>25mm of Hg by transthoracic echocardiogram, Myocarditis defined as unexplained tachycardia(Heart rate > 90/min) with LVS3 with or without LV dysfunction by echocardiogram.Patients with incomplete data were excluded. Descriptive statistics for the categorical variables were performed by computing the frequencies (percentages)in each category.

Results: We screened 76 patient records with Dermatomyositis. Median age of presentation was 43(6-58)years,14 were adults and 2 were juvenile(< 18 years).Majority(60% )were females.Median disease duration was 16.2 months. Sixteen patients (21%)had cardiac involvement,nine(11.8%) of them had PAH,three(3.94%) had myocarditis,two had aortic stenosis, Supra ventricular tachycardia and interatrial septal aneurysm were seen in one each.Three patients with PAH had overlap with other connective tissue disease, of whom two had SLE and one had scleroderma. Forty percent of patients had gottron's papules,33% had heliotrope rash. Arthritis was seen in 58.6%, proximal muscle weakness in 96% and 12% had ILD. Average CPK was 1062 IU/L, and LDH was 900 IU/L.ANA was positive in 65%,most common pattern observed on immunofluorescence was speckled followed by nucleolar and cytoplasmic.

Conclusion: PAH is the common cardiac manifestation in Dermatomyositis at our centre. Screening of every dermatomyositis patient at presentation is necessary for early diagnosis.


  P156 Category: Scleroderma Top


Ophthalmology to Rheumatology in Systemic Sclerosis from a Tertiary Eye care Centre

Panigrahy B & Padhy T; LV Prasad Eye Institute,Bhubaneswar

Introduction: Almost every rheumatologic disease can affect the eye. Ophthalmological complications can be found in any stage of Systemic Sclerosis. Case no 1 - A 44 year lady school teacher presented with pain, watering both eyes and diagnosed to be Peripheral Ulcertive Keratitis(PUK). Repeated PUK flare not responding to local drops. She gives h/o painful color changes in fingers during cold.Physical Examination revealed no abnormality. Lab investigations showed ANA, Anticetromere antibody strongly positive & DS DNA,U1RNP negative. Diagnosed as Very Early Systemic Sclerosis (EUSTAR).Treated with Calcium channel blocker, Oral Steroid and Azathoprine and doing well. Case 2 - A 48 year lady presented with gross diminution of vision since one month. Ophalmological finding showed diffuse bilateral Exudative Retinal Detachment. She gives h/o polyarthritis 3 years, being treated locally with Sulphasalazine, NSAIDS. Gives h/o painful color changes of fingers. Examination revealed puckering of perioral skin, acrolysis of terminal phlanges of fingers,SJC 20/28,TJC 12/28. CBC,RFT,LFT & TFT - WNL. Raised ESR, CXR PA view reticular opacity Right lower zone, 2 D Echo - No PAH, Good LV function, RF - Negative, Anti CCP antibody negative, HRCT Thorax - ILD, X ray hands - resorption of Right 2nd & 3rd terminal phalenx, Barium meal & X ray - Dilatation of esophagus & Cardia, ANA & Anti topoisomerase antibody strongly positive. MRI brain Showing small T2 & flair hyper intensity with diffusion facilitation in left cerebellar peduncle, suggesting ? Late sub acute Lacunar infract. USG abdomen did not show any abnormality. She was treated with Pulse Cyclophosphamide, Oral Steroid,Vit D, Calcium, Nifedipine. Vision was grossly diminished to PL & PR. She was lost to follow up.

Conclusion: In the first case PUK was a presentation of Very Early Systemic Sclerosis where as in the second case bilateral Exudative retinal detachment leading to blindness was a later presentation not found in literature.


  P157 Category: Scleroderma Top


Posteriortibialnerve Stimulationin The Treatmentof Feacal Incontinence In Ctd

Sudheer Nath Sake, Dharmanand BG; Sakra World hospital, Bengaluru, Karnataka

Aim: Fecal incontinence is reported to occur in up to 1/3rd of scleroderma patients. Usually it is treated with dietary modifications and loperamide with partial success. Sacral nerve stimulation has been successful but is cumbersome and may need invasive procedures. Posterior Cutaneous Nerve stimulation (PCNS) was also reported to be successful in reducing the symptoms.We report the use of PTNS using easily available TENS machine in the treatment of fecal incontinence in CONNECTIVE TISSUE DISORDERS (CTD).

Method: Two patients with scleroderma and one patient with SLE with past history of transverse myelitis were treated with posterior tibial nerve stimulation using TENS, for 2 hours a day for three months. The outcome was measured using visual analog scale and bowel dairy.

Results: Two women with Scleroderma were followed up for a mean of 16 months. The faecal incontinence improved up40% in 3 months. The number of incontinence episodes dropped within 2 months of starting the treatment. They have opted to continue the treatment for the perceived benefit. One young lady aged 23 with SLE with residual myelitishad 30% improvement in first 3 months and continues to use the treatment and has been experiencing beneficial results.

Conclusion: Posterior Tibial nerve stimulation (PTNS) seems to be a promising noninvasive method to treat Faecal incontinence, particularly in patients with scleroderma. It can be done at home by patients after a brief period of training. Commonly available TENS machine could be used and is economical. Further evaluation using larger number of patients and using standard assessment tools is required to determine its place in the treatment of faecal incontinence.


  P158 Category: Scleroderma Top


Systemic Sclerosis overlapping neuromyelitis optica - An uncommon association of uncommon disease

Nahar Naisar, Nahar Prachi; Arham Rheumatology Center, Nasik, Maharashtra

Background/Purpose: Neuromyelitis optica (NMO) is an autoimmune disorder and is characterized by relapsing demyelinating disease of central nervous system. It is associated with optic neuritis, transverse myelitis involving three or more contiguous spinal cord segments and seropositivity for NMO-IgG antibody. The term "neuromyelitis optica spectrum disorder" (NMOSD) refers neuromyelitis optica features occurring in association with other systemic rheumatological disorders. Systemic sclerosis (SSc) is also an autoimmune disorder characterized by excess collagen deposition in the skin and internal organs. The coexistence of systemic sclerosis and NMOSD is extremely rare.

Methods: We describe sporadic association of systemic sclerosis with NMO. Data was extracted from our database & case record forms are used to enter patient profile.

Results: 26 year lady admitted for second episode of paraparesis with optic neuritis. She also had history of cerebral venous sinus thrombosis. Rheumatological consultation had taken for cramps. Very interesting facts came obvious after taking detail history. On questioning, found to have raynaud's phenomenon with digital ulcers since 3 years. She also gave history of skin thickening all over the body since 3 years. Her serum was positive for anti-nuclea anti-centromere & anti aquaporin 4 antibody. She was initiated on 1mg/kg prednisolone and azathioprine. She improved and on regular follow up since past 1 yr.

Conclusion: To our knowledge this is the first case report from India to illustrate association of SSc and NMO. Clinician should be aware of systemic sclerosis in patient with neuromyelitis optica spectrum disorder even if they do not complain skin symptoms.


  P159 Category: Scleroderma Top


Neuromyotonia: A Rare Presentation In A Case Of Systemic Sclerosis

Vaijayanti Lagu-Joshi, Sujit Jagtap, Smita Upadhye; Deenanath Mangeshkar Hospital and Research Center, Pune

Background: Neuromyotonia is a rare autoimmune channelopathy. There are very few case reports of neuromyotonia in connective tisssuue disorders. We are presenting a case of scleroderma with neuromyotonia, a rare combination.

Materials And Methods: 44 yrs old male presented with polyarthralgia, dry cough and dyspnoea on exertion over 2 months. Detailed evaluation showed skin changes of scleroderma, active arthropathy and presence of interstitial lung disease(ILD). Laboratory investigations- Rheumatoid factor(RF), Anti-nuclear antibody (ANA), Pm-Scl antibodies were positive. HRCT thorax confirmed ILD. A month later, he started complaining of severe backache and muscle pains with spontaneous and persistent muscle twitching in calves, thighs and arms. He had severe insomnia and restlessness. Electromyography (EMG) done was classical of neuromyotonia and voltage gated potassium channel antibodies (VGKC) were positive. The electrolytes, calcium, CPK were within normal limits, PET scan was normal.He did not respond to oral membrane stabilizers and was given intense immunosuppression with solumedrol pulse followed by 7 cycles of plasmapheresis. He responded well to the therapy in a months time.

Discussion: Neuromyotonia has varied clinical profile with unpredictable natural history. Antibodies against voltage gated potassium channels are often demonstrated in neuromyotonia. It has been associated with few other autoimmune conditions like localized sclerodema and dermatomyositis. Although rare it is potentially treatable.

Conclusion: Both neuromyotonia and scleroderma are autoimmune. But the significance of association is not known. But immunosuppression seems to have good efficacy in the management of neuromyotonia.

Keywords: Neuromyotonia, scleroderma, VGKC antibody, plasmapheresis.


  P160 Category: Scleroderma Top


Scleroderma and  Vogt-Koyanagi-Harada syndrome More Details a rare association

Narendra V1 , Sravan K A1 , Vara Prasad I R2 , Liza Rajashekar3 ; Nizam's Institute of Medical Sciences .

Introduction: Ocular inflammation in scleroderma is rare. Vogt-Koyanagi-Harada syndrome (VKH) is a cell-mediated autoimmunedisease, associations with various autoimmune diseases is reported. We present a rare association of VKH with scleroderma.

Case Details: 27 years male, case of scleroderma with myositis, arthritis and hepatitis was on mycophenolate mofetil (MMF) therapy. Patient's MMF was stopped 4 months ago as he was in remission. Now presented with holocranial headache, gradual painless vision loss, aphasia and altered sensorium of 20 days duration. Ophthalmological evaluation revealed severe diminished visual acuity in both eyes, slit lamp examination showed anterior uveitis with festooned pupil in both eyes. Fundus examination revealed exudative retinal detachment in right eye and multiple neuro sensory detachments in the posterior pole in both eyes with hyperaemic disc. There was no evidence of vasculitis or retinitis. Fundus Fluorescein Angiography and Optical coherence tomographywere suggestive of Vogt-Koyanagi-Harada syndrome. During hospital stay he had persistent altered sensorium with 1 episode of seizure. On further evaluation CSF showed raised protein (288mg/dl), no cells, no organisms isolated on cultures, MRI brain - T2/flair hyperintensities in cerebellum, medulla pons, thalami, and fronto parietal lobes. As his ocular and CNS features are consistent with complete VKH, he was treated with methyl prednisolone pulses for 3 days followed by 1mg/kg steroid. His sensorium improved, vision normalised. He is on regular follow-up for 4 months, continued on MMF as immunosuppression with no relapse.

Conclusion: VKH is a rare ocular autoimmune disease. Association with Scleroderma is reported only once till date. In this rare association interestingly VKH occurred after stopping immunosuppression and responded well to reinitiating of therapy.


  P161 Category: Miscellaneous Top


A case series, Diversity of Pulmonary Hypertension in connective tissue disorder

Manohar Joshi, Rohini Samant, C Balakrishnana, Girish K, R Shah, Parikshit S. ; Hindhuja Hospital, Mahim, Mumbai

PH is defined as an increase in mean pulmonary arterial pressure ≥25 mmHg at rest as assessed by right heart catheterization. WHO Classifications of Pulmonary Hypertension are Pulmonary Arterial Hypertension, Pulmonary Hypertension owing to left heart disease, PH Secondary to Chronic Hypoxemia (LUNG DISEASE), Chronic Thrombo-Embolic Pulmonary Hypertension, Miscellaneous (usually extrinsic compression of pulmonary arteries). A 33 yr old female diagnosed case of RA with DOE Grade I-II (PASP-60mm of Hg) and progressed with DOE Grade-III (PASP-124mm of Hg) on presentation to us. No evidence of ILD on HRCT, No evidence of pulmonary thromboembolism on pulmonary angiography. Two dosages of Rituximab 1gm was given at 15 days interval 2D-ECHO repeated after 4 month shows PASP 103 mm of Hg. A 29 yr/female diagnosed as SLE delivered DOE Grade II and gangrenous changes at tip of great toe. ECHO shows PASP 80 mm of Hg. APA, Anti B2 Glob & ACL is negative, Lupus anticoagulant is absent. Now ECHO showing PASP 52 mm hg, after giving IV Methyl prednisolone and IV cyclophosphamide along with Plasmapharesis. 57yrs / F admitted with h/o glomerulo-nephritis(nephrotic syndrome) since 12 yrs on treatment developed progressive DOE grade IV for last one year, proximal and truncal muscle weakness, GERD, Raynauds, Pedal oedema s/o RV failure. HRCT suggestive of ILD and 2D Echo showing PASP 74 mm Hg. Diagnosis was MCTD, myositis, PAH, Vasculitis. On treatment presented with 4 episodes of massive hematemesis and Severe PAH (PASP- 76 mmHg), Upper GI endoscopy - 3 esophageal varices; Esophageal variseal ligation done, USG abd- Ascites, coarse echotexture of liver, mild splenomaegaly. Final diagnosis was MCTD Probable portopulmonary hypertension (vascular PAH aggravated by portal hypertension), CLD, ESRD on HD A 43 y/F, MCTD with ILD on immunosuppressants treatment was relatively well. Presented with h/o fever with generalized weakness, difficulty in standing up from the sitting position,tachypnea, cough. 2D echo was suggestive of PASP = 64 mm Hg,moderate PAH. HRCT chest: NSIP pattern with BOOP. D dimer was positive. Lupus anticoagulant negative, ACLA positive, CTPA S/O left lower lobe pulmonary embolism and was started on anticoagulation.

Conclusion: Different type PAH presentation can be seen. SLE is rarest among all the CTD as a cause still can lead to it. Even portal hypertension can lead to precipitation of PAH. Early intiation of treatment can lead to recovery, on contrast delayed treatment leads to persistant PAH.


  P162 Category: Miscellaneous Top


IgG4 related disease - patient profile and disease patterns

Bhandari Gurbir Singh, Lalit Duggal, Neeraj Jain, Nagma Bansal, Jeet Patel; Sir Ganga Ram Hospital, New Delhi

Background: IgG4 related disease is a newly recognized, rare fibroinflammatory condition that can involve various organ systems. The aim of this study was to identify the different clinical patterns of this disease, in a tertiary care centre.

Methods: Patients were diagnosed on the basis of published diagnostic criteria for IgG4 related disease. We recorded their presenting complaints, epidemiological profiles, laboratory, radiological and histological findings along with the treatment and outcomes.

Results: We diagnosed 16 cases of IgG4 related disease from 2012-2015. 14 had involvement of orbits and peri-orbital tissues, 2 had lymphadenopathy, involvement of retroperitoneal tissue, aorta and paranasal sinuses was seen in 1 case each. 4 patients had involvement of multiple organ systems. Overall Female to Male ratio in our study was 1.7:1, but this ratio increased in those with multi-organ involvement. Majority of the patients were above the age of 50 years at the time of diagnosis. Serum IgG4 levels were elevated in 58.3% cases with single organ involvement, while all the patients with multi-organ involvement had increased IgG4 levels. The number of cases diagnosed as definite, probable and possible IgG4 related disease according to diagnostic criteria were 8, 5 and 3 respectively. CRP levels in patients with multi-organ involvement were >3 times upper limit of normal. All patients were treated with steroids to begin with. Azathioprine was used as a steroid sparing agent. 2 patients with resistant disease were given rituximab. Overall response to treatment was good.

Conclusion: This study on IgG4 related disease conducted in our rheumatology department over a 3 year period depicts the most common patterns of organ involvement along with the epidemiological, laboratory, histological, radiological data and response to treatment.


  P163 Category: Miscellaneous Top


Old Wines In A New Bottle: Case Series Of IgG4

Paul Antony T, Antony T P1 , Jayaraj K1 ; Departments of Immunology,1 General Medicine, Amala Institute of Medical Sciences, Thrissur

Background/Purpose: IgG4 related diseases(IGG4RD) are a relatively new terminology introduced to reclassify many of the previously diverse disease groups. Although initially described in Japan, reports of its prevalence from other parts of the world are being acknowledged. There is not much data available from our part of the world.

Methods: Patients satisfying the international consensus criteria for diagnosis of IgG4 related disease were enrolled in the study. A predesigned Performa for analysing the clinical details and examination findings was made. In all the patients routine blood hemogram, ESR, CRP, renal and liver function tests were done. Imaging as considered per case was done. All cases were assessed for their immunoglobulin (IgG, IgE, IgG4) levels. Histopathology with IgG4 staining was done in all cases where tissue was accessible.

Results: Fifteen patients were included in the study. There was a slight female preponderance. The mean age of patients in the study was 52 years. The sites of involvement were variable and included meninges, salivary gland, pancreas. 85% cases had elevated IgG4 levels. Around 65% cases had elevated IgG levels. Typical histopathology was detected in all our cases.IgG4 staining was positive in 70% of the sent cases. All patients were treated with oral corticosteroids with majority responding at a prednisolone equivalent dose of 20 mg. Methotrexate was the most commonly used steroid sparing agent.

Conclusion: IgG4 related disease should be considered as differential diagnosis in cases especially when the patient is on the older age group and has a mass. Treatment is very rewarding for the physician.


  P164 Category: Miscellaneous Top


Beware of Bugs: a case series

Kakade Girish, Samant Rohini, Sagdeo Parikshit, Shah Romi, Joshi Manohar; P D Hinduja National hospital & Medical Research Centre, Mumbai

Case 1: 22 years male, k/c/o ulcerative colitis, developed insidious onset, bilateral knee pain and swelling. Rheumatologist treated him with sulphasalazine, oral & intraarticular steroids, f/b infliximab. He worsened with treatment. On presentation he had bilateral knee swelling, 90 flexion deformity. X -ray and MRI showed gross destruction. He underwent bilateral knee synovectomy. Culture grew salmonella species.



He didn't improve after 6 weeks of antibiotics. Surgical debridement was planned, but he didn't follow up.

Case 2: 62 years diabetic female, presented with 15 days h/o multiple joint pain & swelling, fever on first day. She had loose motions 3 weeks prior for 2 days. She had bilateral shoulders, right knee & left MTP synovitis. She was started on NSAIDS. On third day, she had fever with altered sensorium. MRI showed bilateral acute frontal infarcts with meningeal enhancement. Blood, CSF & pus cultures from subsequent left foot abscess grew streptococcus agalactiae. She improved after 6 weeks antibiotic therapy.

Case 3: 50 year diabetic female, k/c/o rheumatoid arthritis on DMARDS, was admitted outside with 3 months h/o severe right hip pain. She was given oral steroids without improvement. During hospital stay, she developed fever, hypotension & dyspnoea. Blood culture grew MSSA. She improved with 1 week of IV antibiotic. On presentation, she had severe hip pain with reduced movements in all directions. X-ray showed extensive hip damage. Aspiration of fluid suggested pyogenic infection. Culture grew MSSA. She improved significantly with excision arthroplasty & 6 weeks of IV cefazolin.



Discussion: In patients with inflammatory arthritis with recent mono/oligoarticular flare, not responding to stepping up treatment, infection should be ruled out. Delayed diagnosis & treatment leads to irreversible loss of joint function.

Conclusions: High suspicion is required for diagnosis of infection in inflammatory arthritis.

If doubtful, synovial fluid and blood cultures should be sent.


  P165 Category: Miscellaneous Top


An Asplenic Patient Presenting With Fever And Polyarthritis - Case Report

Ramadoss Ramu, Vivek Arya, Rajesh S Taneja; Department of Medicine, PGIMER & Dr. Ram Manohar Lohia Hospital, New Delhi

Background: Adult onset Still's disease (AOSD) is a rare inflammatory disorder of unknown etiology with a prevalence of less than 1 case per 100,000 people. It is a diagnosis of exclusion and considered only after other common etiologies have been excluded. Its main features are high grade fever, polyarthralgia, evanescent rash, lymphadenopathy, hepatosplenomegaly and elevated liver enzymes, ferritin and raised ESR.

Case Presentation: A 39 year old man presented to our hospital with complaints of high grade fever, joint pain and body ache for one month. He had swelling of the right knee and small joints of the hands. There was history of splenectomy 10 years ago following blunt injury to the abdomen. Since Overwhelming Post Splenectomy Infection (OPSI) was the initial probable diagnosis, empirical antibiotic therapy was initiated and symptomatic treatment for joint pain was given. Evaluation to find a septic focus and autoimmune diseases was carried out which showed negative results. There was no improvement in symptoms after a week of treatment. Subsequent investigations showed persistently elevated total leukocyte count with neutrophilic predominance, raised liver enzymes, ESR and CRP levels. Notably serum ferritin levels were markedly elevated (3230 ng/ml). Since alternative diagnoses (infections, autoimmune diseases and malignancies) were excluded and the Yamaguchi's criteria for AOSD was fulfilled, the patient was treated with IV steroids which resulted in rapid resolution of his symptoms. He was discharged on 1 mg/kg of oral prednisolone and 15 mg/ week of methotrexate. Patient is doing well on follow up visits.

Conclusion: The presentation of fever with asplenia initially made us focus on OPSI. But when the patient did not improve with initial management, we considered alternative diagnoses thus arriving at the rare diagnosis of AOSD. Keeping an open mind while evaluating the patient can prevent an unusual disease from being misdiagnosed.


  P166 Category: Miscellaneous Top


Amyloidosis- Case series from a tertiary care hospital

Srinath R1 , Hitha B2 , Amalnath DS1 , Wyawahare M1 , Subrahmanyam DKS1 , Rajesh NG3 ;1 Department of Medicine,2 Department of Clinical Hematology,3 Department of Pathology, JIPMER, Puducherry

Background: AL amyloidosis is a plasma cell dyscrasia with multisystem involvement due to the deposition of immunoglobulin light chains. It is often missed clinically and underdiagnosed on biopsy. Prompt diagnosis and early treatment may improve survival of patients.

Methods: Four patients were identified in our department within 18 months from 2014 to 2016.

Results: Age, gender and clinical findings in patients are tabulated below. Three patients presented with fluid overload. Investigations revealed nephrotic proteinuria in all three patients, Bence-Jones proteinuria and elevated alkaline phosphatase in two patients. Electrocardiogram showed low voltages but echocardiography revealed concentric LV hypertrophy and 'sparkling myocardium' in all three patients. This unusual combination was a strong pointer towards amyloidosis. Further tissue diagnosis was done as tabulated below. The fourth patient presented with isolated inguinal lymphadenopathy. Lymph node biopsy showed amyloidosis and bone marrow revealed IgG myeloma. All patients fulfilled the Mayo clinic/IMWG diagnostic criteria. Bortezomib based therapy was started for patient 1 (two cycles completed) and melphalan plus dexamethasone in patient 2. Patients 2 and 3 expired due to refractory cardiogenic shock. Patient 4 opted out of treatment.

Conclusion: The diagnosis of primary AL amyloidosis requires a high degree of clinical suspicion. Early diagnosis and treatment may improve overall survival.




  P167 Category: Vasculitis Top


An aetiological and clinicopathological study of Cutaneous vasculitis - A morbid experience!

S. Santhanam1 , S. Samuel2 , M. Vij3 , N. Singh2 ;1 Rheumatology,2 Dermatology,3 Pathology, Global Hospitals, Chennai, India.

Background: Cutaneous vasculitis has a varied presentation with various aetiologies. It can be the initial presenting manifestation of many of the rheumatological disorders and needs an extensive workup to ascertain the cause and to look for extracutaneous involvement. The management is quite challenging due to increased chances of relapses.

Objectives: To study the aetiology, extracutaneous manifestations, biopsy findings and treatment modalities in patients presenting with cutaneous vasculitis.

Methods: 51 consecutive patients who presented to the rheumatology department (Oct 2014 - Mar 2016) with features of cutaneous vasculitis were included in this cross sectional study. A detailed history, clinical examination and all relevant investigations (including skin biopsy) were done. Histopathological examination was done in 39 patients and immunofluorescence study was done in 28 patients.



Results: Out of 51 patients, 18 of them were classified as hypersensitivity vasculitis, 7as lupus, 7 as isolated cutaneousleukocytoclasticangitis, 6as rheumatoid vasculitis, 2 each as Sjogren's syndrome, dermatomyositis, cutaneous polyarteritisnodosa, erythema induratum and adult onset henochschonlein purpura. 1each were classified as ANCA associated vasculitis, Cryoglobulinemic vasculitis and Urticarial vasculitis. 32 patients were females and rest males. Twenty eight had fever, 14 had arthritis, 7 had proteinuria, 3 had neurological involvement, 3 had myositis, 2 had hematuria and 1 had vasculitis of small intestine. Thirteen patients had relapses during treatment. Out of 39 patients who had skin biopsy, 35 had leukocytoclastic vasculitis, 2 had pan arteritis and 2 had panniculitis with vasculitis. For treatment, steroids were used in 33 patients, mycophenolate mofetil in 11, azathioprine in 8, methotrexate in 6, cyclophosphamide in 2 and colchicine in 2patients.

Conclusions: In our study, though only few had a major organ involvement, the majority had frequent relapses. Hence, they were treated with steroids and other immunosupressivesfor a extended period. To conclude, cutaneous vasculitis has to be investigated extensively and treated adequately to prevent relapses and hence to prevent morbidity.


  P168 Category: Vasculitis Top


A study of clinical profile in takayasu arteritis

Dr N. Ezhil, Dr P S Arul Raja Murugan; Govt Rajaji Hospitalmadurai, Tamilnadu

Background: Takayasu arteritis also known as pulseless disease or occlusive thromboaortopathy is a vasculitis of unknown cause, with wide array of clinical presentations starting from being asymptomatic to life threatening organ damage. This study is aimed at learning the distribution, clinical profile, presenting complaint, correlation with acute phase reactants and the angiographic categorization in a tertiary care hospital.

Materials and Methods: This is a retrospective observational study conducted in a tertiary care hospitalbetween 2014-2016. 35 patients satisfying ACR criteria for takayasu arteritis were taken into our study comprising 22 females and 13 males.



Results: All 35 patients had absent pulses at presentation. We were not able to pick up the disease in early stages. All were <40 yrsof age except 4. 13 patients had CVS involvement, 12 had multi system involvement, 8 had CNS involvement and 2 had renal involvement at presentation. The most common presenting complaint being claudication. ESR was elevated in 27 patients, CRP was positive in 23 of them.


  P169 Category: Vasculitis Top


Topical Etanercept for Inflammatory Ulcers- A case Series

Dr. VikramHaridasand, Dr. Praveenkumar Shetty; Rheumatoid arthritis superspecialty center, Hubli and SDM College of Medical Sciences & Hospital, Dharwad

Background: Vasculitis is an inflammation with destruction of the vascular wall. It appears as an idiopathic form as well as one associated with an underlying autoimmune disorder. This can also be triggered by certain drugs prescribed to these autoimmune conditions. The first line of treatment for this vasculitis is systemic corticosteroids and immunosuppressants. Use of anti TNF-α therapy has broadened the therapeutic option.

Methodology: In this case series we are demonstrating the successful topical treatment of representative vasculitis cases with etanercept, which was not responding for the regular regimen.We collected biopsy of the wound before and after two months of treatment and analyzed for local inflammatory cytokines and their upstream molecular markers and tissue hydrolyzing enzymes.

Result and Conclusion: With all the steroid and immunosuppressive back ground treatment subcutaneous injection of less expensive etanercept was treated, once it did not show any improvement, topical application of this TNF alpha blocker was used successfully initially in one PG. The same treatment was successfully confirmed in many vasculitis and bachets disease. IHC analysis demonstrated the role of TNF α and its associateother cytokines and upstream molecular markers and other tissue hydrolyzing enzymes in the vasculitis pathogenesis.Usually vasculitic ulcer cases will be associated with other autoimmune conditions, so systemic treatment will not give the expected results. In this respect topical treatment of this TNF blocker will be a land mark treatment option in these types of patients. Significant clinical result, which is supported by the decreased expression of inflammatory markers like AnxA2, MMP-9 and IL-6 at the wound site and increase in anti-inflammatory AnxA1 expression after the treatment reveals that wound microenvironment is devoid of all inflammatory molecules and suffice the reason to believe that topical treatment of TNF α blocker is the way ahead in the treatment of vasculitis.


  P170 Category: Vasculitis Top


Relapsing polychondritis associated with pyoderma gangrenosum, a rare presentation: A case report

Arjun MN, Vasdev Vivek, Hegde Arun, Choudhury GD, Kumar Abhishek; Army Hospital Research & Referral, New Delhi

Background: Relapsing polychondritis (RPC) is a rare chronic, recurrent, inflammatory disease of unknown etiology, primarily affecting the auricular, nasal,and laryngotrachealcartilages.Pyoderma gangrenosum is a chronic cutaneous disease that usually presents as a painful nodule or pustule, progressively forms an enlarging ulcer and is rarely associated with RP.Diagnosis is based on McAdamcriterion. Corticosteroid remains the mainstay of treatment along with the anti-inflammatory drugs.

Case Summary: This 58years old male presented with recurrent episodes of polyarthritis involving metacarpophalangeal (MCP) joints, wrist, knee, ankle joints and intermittent episodes of fever, redness of eyes, pustules over dorsum of right hand and legs. Few months ago he had self-limiting recurrent episodes of pain and swelling of ear and nasal cartilages, each episode lasted for 2-3 weeks. Clinically both ears were swollen, tender, red and warm sparing ear lobe. There were few well circumscribed pus filled lesions over dorsum of right hand and left leg.Investigations revealedHb% 8.2 gm/dl, PBS normocytic and normochromicanemia, raised ESR (140 mm 1st hour), ANA/ANCA by (IIF) were negative, RF/Anti CCP were negative. Hypoalbuminemia (Alb-2.2 g/dl, total protein- 8.2 g/dl), Serum protein electrophoresis A:G ratio normal, no 'M' spike seen. Biopsy of pustular lesion was consistent with pyoderma gangrenosum. A final diagnosis of RPC was made. Patient fulfilled four McAdam's criteria including a history of auricle, nasal chondritis, seronegative nondestructive arthritis, skin lesions and ocular inflammation.The patient was given prednisolone 50 mg daily on tapering doses. A significant improvement in the patient's condition was observed, skin lesions were resolved, and joints swelling and pain subsided.

Conclusion: Relapsing polychondritis is relatively uncommon disease, which explains why the diagnosis is often delayed. Additionally, this is a rare case of RP associated with pyoderma gangrenosum and greater awareness will ensure early detection and treatment


  P171 Category: Vasculitis Top


Multiple cranial nerve palsies associated with Takayasu's arteritis

Saumya Ranjan Tripathy, Anupam Wakhlu, Puneet Kumar, Durgesh Srivastava, Meha Sharma, Danveer Bhadu; King George's Medical University, Lucknow

Background/Purpose: Takayasu's arteritis is a large-vessel vasculitis affecting the aorta and its main branches. Cranial neuropathy is rare and infrequently reported.

Case History: A 20year female developed non-migrainous headache followed by hoarseness of voice and dysphagia to both solids and liquids, which improved with indigenous medications over 2months. 4years later she developed 2nd episode of headache followed by diplopia on right-gaze, treated with indigenous medicines and recovered in 3months. She developed 3rd episode of headache, associated with difficulty in closing right eye, deviation of angle of mouth and tongue to the left. Due to incomplete relief after 6months of local medicines, she presented in neurology OPD. Right facial-nerve and left hypoglossal-nerve palsy was confirmed. Her MRI-brain with contrast, MR-Angiography of brain and CSF-analysis was normal. She was referred to Rheumatology for ruling out CNS-vasculitis. History revealed bilateral lower-limb and right upper-limb claudication for last 4years. Right upper limb and bilateral lower limb pulse were undetectable. CT-Aortography and its major branches revealed right subclavian artery mural thickening with severe luminal stenosis, involving the origins of right internal thoracic artery and right vertebral artery and long segment mural thickening and stenosis of infra-renal aorta extending upto the origin of bilateral common iliac arteries consistent with Takayasu's arteritis. ESR (Westergren) was 100mm 1st hour. She was started on oral prednisolone 1mg/kg and methotrexate. After 3months, her claudications had significantly reduced and eye closure was normal. She had residual left-sided tongue deviation.

Conclusion: She probably had 9th and 10thcranial nerve palsies in her 1st episode, right 6thnerve palsy in 2ndepisode and right 7th and left 12thnerve palsy in her last episode. Although the exact cause could not be determined due to late presentation and treatment modified disease status, the case highlights that Takayasu's arteritis can have multiple cranial nerves palsy as a rare presentation.


  P172 Category: Vasculitis Top


Topical TNF-α inhibition - A novel therapy for vasculitic ulcers in Behcet's disease

Natasha Faria, Srinivas C, Shailee Chandak, B G Dharmanand; Sakra World Hospital, Bengaluru

Background: Behcet's disease is a systemic vasculitis of unknown etiology that ischaracterized by recurrent mucocutaneous manifestations and chronicrelapsing uveitis. Cutaneous ulcers are a relatively rare manifestation, for which there are no current treatment recommendations. Systemic treatment with Azathioprine, Cyclophosphamide or TNF-α antagonistsmay be used for refractory cases. We report the use of topical Etanercept for a non-healingcutaneous ulcer in Behcet's disease.

Case Report: Our patient is a 23-year-old man, who was diagnosed with Behcet's disease 4 years ago. He has been on regular treatment with oral prednisolone and Azathioprine. He presented 4 months ago, with erythema nodosum like lesions on the right foot, which gradually ulcerated and coalesced to form an ulcer over the dorsum of the foot, measuring 13cm x 8cm. He was initially treated with Colchicine and regular dressings of the ulcer along with steroids and azathioprine. However, there was no improvement. Skin grafting at this stage was not possible because of unhealthy granulation tissue and the size. Cyclophosphamide was deferred in view of his age.An Etanercept biosimilarwas applied over the ulcer every 3 days;5 applications over 2 weeks. The dose of oral prednisolone was decreased and Azathioprine was continued. This resulted in the development of healthy granulation tissue by Day 12. After this, regular dressings were continued. A split thickness skin grafting was done after another 3 weeks. The patient was followed regularly over two months and the graft has taken well.

Conclusion: Borrowing from the anecdotal report of similar success from a colleague, we report using Etanerceptlocally as an alternative to systemic therapy, and thereby reducing the cost burden to the patient and minimizing potential adverse effects. A larger case series is warranted.


  P173 Category: Vasculitis Top


Pyrexia of unknown origin without headache- Could this be GCA?

D Ramdin, S Jagadeesh, P Das, A Kuttikat; Kettering General Hospital NHS Foundation Trust

Introduction: We report an atypical and unusual presentation of a multisystem condition that intrigued medical specialist including respiratory and gastroenterology specialists.

Case Summary: A 67 year gentleman (20 pack year ex-smoker) was initially referred to the respiratory clinicby the GP for suspected lung cancer as he haddry cough, fever, fatigue, reduced appetite& unintentional weight loss of 1/2 stone over 8 weeks. Patient denied shortness of breath or chest pain. He had raised inflammatory markers (ESR-132, crp-282). CT Chest was normal. However, given his loose bowel motions, deranged liver function (ALT 194, ALP 354), Iron deficiency anaemia, he was referred to gastroenterology who ruled out any GI cause (normal CT abdo & Pelvis, negative hepatitis screen). He was admitted with worsening symptoms and reported leg weakness & urinary retention and transient visual blurring (10 minutes). Echocardiogram, Blood & urine cultures, EBV, CMV, HIV, lymphoma screen, CT head, MRI Spine were all normal. A course of antibiotics did not impact on symptoms or high inflammatory markers. Ophthalmology review was normal and they did not find any evidence of GCA. He was then referred to Rheumatology and on review; he denied headache, visual loss, jaw or tongue claudication but reported mild proximal girdle stiffness. There was no scalp tenderness & his temporal arteries were pulsatile and non-tender. ANA and ANCA, DNA, ENA, anti CCP and rheumatoid factor all were negative. Large vessel vasculitis was suspected and this was confirmed on FDG-PET scan &temporal artery biopsy confirmed classic GCA histological features. He was started on oral prednisolone and had an excellent response with resolution of all of his symptoms and biochemical parameters.

Learning points

  • GCA can very rarely present without any significant headache.
  • GCA can occasionally present with neurological and respiratory symptoms. (1)
  • GCA can cause significant abnormalities in LFT.
  • FDG-PET scan can be extremely useful in diagnosing GCA with large vessel involvement.



  P174 Category: Vasculitis Top


Design and evaluation of cryodevice, an easy to use apparatus for maintenance of temperature for cryoglobulin assay

Praveen Krishna V1 , Jayakanthan Kabeerdoss2 , Pulukool Sandhya2 , Suresh Devasahayam1 , Debashish Danda2 ;1 Department of Bioengineering,2 Department of Rheumatology, Christian Medical College, Vellore

Background/Purpose: Maintenance of temperature is integral to cryoglobulin assays. Potential false negatives could occur when blood temperature drops below 37°C. To maintain the temperature, warm water bath or aluminium foil are commonly used though these may not maintain a consistent temperature. We describe the design and evaluation of a low-cost device "cryodevice" that could protect blood samples from thermal fluctuations during transportation from phlebotomy room to laboratory.

Methods: The design encompassed a double-walled vessel. The inner wall was made of copper and the outer wall of insulating PVC. Between the walls, an electrically-powered heating coil was placed in heat retaining liquid water, which provides even heating (Fig 1a, 1b & 1c). The temperature was controlled precisely using a thermistor for sensing and an electronic circuit for bang-bang feedback control. Required power was 12V DC. The device was adequately insulated with a wooden sleeve and polyurethane foam. Computer simulations using Solidworks® were used to plot the expected operational ranges of temperature fluctuations.

Results: The cryodevice was equipped for preheating the vaccutainer tube and temperature maintenance during storage, transport and incubation of the blood sample. Computer simulations and in vitro testing forecasted satisfactory performance with regard to temperature maintenance (Fig 1d). Over 1 hour, the temperature drop was not more than 1.5°C when taken off power. Evaluation of the device in clinical settings revealed that the temperature was maintained at 37±0.5°C over several hours of monitoring during incubation. The device was tested on 45 samples in last 8 months. One each with primary Sjogren's syndrome, hepatitis C infection and Raynaud's phenomenon tested positive. The device was easy to handle, weighed under 500 gm and costed around ₹800.

Conclusion: Cryodevice is a cost effective, easy to build and handy device that can be used for stringent temperature maintenance during cryoglobulin assays.


  P175 Category: Vasculitis Top


An unusual case of Childhood Polyarteritis Nodosa

Kishore Kunal, Hegde Arun, MN Arjun, Vasdev Vivek, Kumar Abhishek, Choudhury GD; Army Hospital Research & Referral, New Delhi

Background/Purpose: Polyarteritisnodosa (PAN) is a rare systemic vasculitis characterized by necrotizing arteritis of predominantly medium sized vessels leading to micro-aneurysms, rupture and hemorrhages, and manifesting with multisystem involvement. Childhood presentation is rare and very few cases have been reported from India.

Case Report: This 16years old girl became symptomatic in May 2007with unexplained hypertension detected during pre-operative evaluation for subacute intestinal obstruction. Evaluation for secondary causes of hypertension was negative, and she was started on oral anti hypertensives with fair control of blood pressure. She again became symptomatic in 2012, with insidous onset Lt heel enthesitis, dactylitis of Lt great toe and arthritis of small joints of Rt hand of 6 months duration. Evaluation revealed a normal haemogram, elevated acute phase reactants, negative ANA by immunofluorescence, along with a positive HLA-B27 status. She was also incidentally detected HbsAg positive, with normal HBV DNA levels. She was diagnosed as a case of Juvenile Idiopathic Arthritis (Enthesitis related Arthritis) and started on DMARDs and NSAIDs with initial improvement in symptoms. However she again became symptomatic after 6 months with fever, Rt heel enthesitis and poor blood pressure control. She was re-evaluated for her disease, and digital subtraction angiography (DSA) revealed numerous microaneurysms in hepatic and renal distal small arteries. Her ANCA(IIF) and Anti MPO/PR3 ELISA were negative. She however had elevated HBV DNA titres. She was diagnosed as a case of Childhood Polyarteritis Nodosa and managed with antivirals, followed bysteroids and DMARDs. Follow up DSA at 1 yr showed complete resolution of microaneurysms.

Conclusion: Childhood PAN can be challenging to diagnose and a high index of suspicion is requiredin these cases. Most children have a monophasic course and respond to steroids, but some children go on to require DMARDs and biologic therapies.


  P176 Category: Vasculitis Top


Study of clinical profile of Macrophage activation syndrome in rheumatology patients of a tertiary hospital and assess the applicability of the criteria of HLH 2004 and Macrophage activation syndrome (MAS) 2016.

Harish Kumar M1 , Vara Prasad IR2 , Liza Rajashekar3 ; Nizam's Institute of Medical Sciences

Objective: To describe the clinical and laboratory features of MAS and to assess the applicability of HLH 2004 and MAS 2016 classification criteria in rheumatic patients.

Methods: Case files of all inpatients diagnosed with MAS during 2014-2016 were identified and clinical, laboratory, treatment details and outcomes were noted.These parameters were matched with HLH 2004 and MAS 2016 criteria and patients who fulfilled all the criteria were considered to have definite MAS and those who fulfilled partially were excluded.

Results: A total of 11 patients were identified.They had a median age of 22yrs(11-45). The rheumatic diseases identified are SLE(8/11), SOJIA(1/11), scleroderma(1/11) and viral (EBV induced) myositis(1/11). The most common clinical presentations were high fever(11/11) and arthritis(10/11). One patient had seizure and cognitive dysfunction.Laboratory findings showed median values of Hemoglobin 7.8(6.1-11.8gm %), leucocytes 3600(200-25100/mm3), platelets 64000(10000-130000/mm3), AST 246 (36-783IU), ferritin 1509(549-1942mcg/litre), triglycerides 369.5(236-463mg/dl), bone marrow hemophagocytosis (5/11). All patients were treated with pulse methyl prednisolone followed by maintenance and only three patients required cyclosporine for recovery. Two patients died. The remaining patients had complete recovery and continued regular medication for the primary disease and are under follow up (median 12months). One patient who had EBV induced MAS and myositis has been completely tapered of medication. The sIL-2 antibody and NK cell activity assay could not be done due to high cost and non-availability at our centre. All the patients fulfilled the criteria of HLH 2004 and of MAS 2016 approved by EULAR/ACR.

Conclusion: In our study all the patients fulfilled the criteria of HLH 2004 and of MAS 2016 approved by EULAR/ACR with SLE being the commonest underlying condition and most of them showed response to pulse steroid therapy. A diagnosis of MAS should be considered when a patient with rheumatic disease presents with high fever, cytopenias and transaminitis.


  P177 Category: Miscellaneous Top


Profile Of Crescentic Glomerulonephritis Patients From A Tertiary Care Centre

Dr. Atanu Pal1 , Dr. K L Karmakar1 , Prof. R. Pandey2 ; IPGME & R, Kolkata

Background/Purpose: Presentation of cresentic GN is rapidly progressive and devastating. It is mandatory to diagnose it at the earliest to save the life and to prevent the end organ damage. There is also scarcity of data. Here we are sharing our experience of crescentic glomerulonephritis (GN) due to vasculitis at our institution.

Methods: We included biopsy proven Crescentic GN patients attending our Nephrology Department.The relevant history was taken, physical examination and investigations were done. They were treated as per standard protocol and outcomes were recorded.

Results: A total of 1023 patients were biopsied due to various renal disorder in one year.35 patients were cresentic GN without SLE. Out of them 64% were female. Most of the patients were between 20 to 60 years of age (average being 37.5 yrs). 71.1% had Pauci immune, 24.4% had immune-complex and 4.4% had anti GBM disease. Among the Pauci immune group,68.75% were ANCA positive and 31.25% were ANCA negative. Among the ANCA associated GN, 31.25% had p-ANCA and 37.5% had c-ANCApositivity. There was a significant delay in the diagnosis. Oliguria was the commonest presentation. Other clinical features like fever, seizures, encephalopathy, rash, arthralgias, and hypertension were present in varying degrees in different groups. Renal dysfunction was obvious in all the groups and average baseline serum creatinine was 12.94 mg/dl in anti GBM group and 5.08 mg/dl in pauci immune group. Daily proteinuria was higher in pauci immune group of 2.84g /d. Nephrotic range proteinuria though not common but it was seen in both ANCA positive and ANCA negative group at diagnosis. Common nonspecific symptoms were fever, otic and nasal discharges which were noted in 62 - 70 % cases. Pulmonary (3.3%) and cutaneous (17%) involvement were other common extra renal manifestation. The patients were treated with steroids, cyclophosphamide and plasmapheresis as per standard guidelines. At the end of three months, 42.68% of patients expired. Those who survived, only 37.14% achieved remission and 68.5% patients were landed in ESRD. c- ANCA positivity had worst outcome.

Conclusion: Renal and patient survival in patients with cresecntic GN due to vasculitis is still very poor. This disease needs further research for better patient outcome.


  P178 Category: Miscellaneous Top


Internal medicine residents' perception of rheumatology as a subspeciality: a web based survey

Keerthi Talari1 , Kirthi Theja Bommakanti2 ; Yashoda Hospitals, Secunderabad

Background/Purpose: Rheumatology is sought as a career option by very few postgraduates. In this study we sought to assess internal medicine residents' perception about rheumatology.

Methods: We conducted a web based survey on awareness in rheumatology among MD and DNB General Medicine residents.

Results: Overall response rate for the survey was 60.7%(48/79). 1/48 was not aware of rheumatology being a DM course. 41 could list institutes offering DM courses. The institutes listed include NIMS (n=41), PGIMER (n=21), AIIMS (n=20), CMC (n=6), JIPMER(n=5), SGPGI (n=2), KMC,Manipal (n=1), SVIMS (n=1). 42/47 felt that there was no adequate rheumatology training time spared during their course. 35 (76.1%) felt conducting regular seminars on clinical management of rheumatology cases by a trained rheumatologist, 40 (87%) felt bedside case discussions by a qualified rheumatologist, 24(52.1%) felt having a rheumatologist in their hospital, 22(47.8%) felt conducting regular CMEs and 11(23.9%) felt making rheumatology case a compulsory case in final examinations could improve rheumatology training. 21.7% were confident of managing rheumatoid arthritis while 78.2% were confident of managing lupus, 23.9% of systemic sclerosis and vasculitis, 23.9% of sjogren's, 8.6% of dermatomyositis, 13.04% of fibromyalgia and 4.3% of soft tissue rheumatism. All the residents felt that there is lack of awareness about rheumatology in the general population and the reasons quoted were rheumatologic diseases are under diagnosed and referred only when the disease becomes disabling (73.9%), no adequate number of rheumatologists in the state(45.7%), no rheumatologists in government hospitals of the state (45.7%), rheumatology cases are manged by other specialities (45.7%), people believe that there is no treatment for arthritis in allopathy (45.7%) and multispeciality coordination in rheumatology is lacking (30.4%).

Conclusion: Despite a compulsory rotation in rheumatology or an in house rheumatologist in the hospital, majority of the internal medicine residents believe that there is inadequate time spent in rheumatology training in their course. Awareness of institutes offering DM course in rheumatology is grossly lacking. Case discussions, regular seminars, rheumatology rotation posting, annual assessment and career training are some options which may improve confidence among residents in rheumatology clinical skills and in choosing rheumatology as their career option.


  P179 Category: Miscellaneous Top


Evaluation of knee joint damage in Hemophilia A using Ultrasonography and its correlation with clinical parameters.

Debanjali Sinha1 , Arijit Nag2 , Sumantro Mondal3 , Shounak Ghosh4 , Alakendu Ghosh5 ;1 Department of Rheumatology, IPGME & R and SSKM Hospital, Kolkata,2 Institute of Hematology and Transfusion Medicine, Medical College, Kolkata,4 Department of Medicine, Medical College, Kolkata,5 Department of Rheumatology, IPGME & R and SSKM Hospital, Kolkata

Background/Purpose: India has the second highest global burden of Hemophilia. 90% of severe Hemophilia A patients have arthropathy. Ultrasonography has emerged as a novel imaging modality in the assessment of Hemophilic arthropathy. Our study aims to evaluate knee joint damage in Hemophilia patients by Ultrasonography and correlate with clinical parameters.

Methods: We studied 27 patients of Hemophilia A and excluded patients who had acute bleed over the past 1 month. The clinical parameters collected were: age, age of onset, severity of haemophilia, total number of bleeds in each knee joint and frequency of Factor VIII infusion. Ultrasonography was done of both their knees. Cartilage (average of medial, central and lateral compartments) and synovial thickness were measured and presence of any vascularity or intra-articular collection noted. Ultrasonography was also done of 27 age-matched male controls and their synovial and cartilage thickness were compared with that of the cases stratifically in 0-10, 11-20 and > 20-year age groups. Correlation study was done between cartilage thickness and the above clinical parameters.

Results: Of the 27 Hemophilic patients, 9 had moderate and 18 severe disease. The mean age of the patients was 16.57 years. The mean cumulative bleeds in right and left knee joints were 47.44 and 33.56 respectively. There was significant increase in synovial thickness and decrease in mean cartilage thickness in the patients as compared to controls across all age groups [Table 1]. There was no significant co-relation between cartilage thickness and age, severity of haemophilia, total number of clinical joint bleeds or frequency of factor requirement. 22.22% (6/27) patients had evidence of intra-articular blood collection despite being asymptomatic.

Conclusion: Premature knee cartilage degeneration occurs in Hemophilic patients, irrespective of the age, severity, number of bleeds or treatment. Ultrasonography can also detect subclinical bleeds that may affect articular outcome in Hemophilia.

Table 1:




  P180 Category: Miscellaneous Top


Effect of Counselling related to tobacco use in systemic immuno-inflammatory arthritides

M. Sadhana Singh Baghel; ARHP(USA)

Background: Inflammatory rheumatological diseases are chronic illnesses where pain, stiffness and increasing disability are common. Tobacco exposure has been shown to be deleterious for these disease requiring counselling aginst it.

Methods: A survey was conducted on the tobacco use status of 211 pts attending the Rheumatology OPD. The F: M ratio was [F 108(51.18%)/M 103 (48.81%)] 1.04:1. The diagnosis among them was as follows: RA in162 pts (76.78%) [101 (62.35%) F and 61(37.65%) M]; SpA in 49 pts (23.22%) [7 (14.28%) F and 42(85.71%) M]. 161 (76.3%) were from the urban and 50 pts (23.7%) from the rural area. Their educational status was as follows; 19 (9%) illiterate, 79 (37.4%) basic education, 64(30.33%) graduate, 49 (23.22%) postgraduate. Tobacco use was categorised as active [smoking or oral tobacco use] or passive (when the patient was exposed to tobacco smoke). Active tobacco users were 74 (35.0%) and passive user were 52 (24.6%).

Results: Total of 126 pts (59.7%) were tobacco users. Counselling intervention to give up smoking yielded the following results: 29 pts (23.01 %) gave up tobacco use, 48(38.09%) did not give up and in 49 pts (38.9%) the information could not be obtained. Out of 74 active tobacco users; 25 (33.78%) had given it up, 40 (54.05%) did not give up, in 9 (12.16%) pts the information could not be obtained. Out of 52 passive smokers; 4 (7.7%) had given it up, 8 (15.38%) did not give it up, in 40 (76.92%) the information could not obtained. Out of 74 active tobacco users, 68 (91.9%) were M, 6 (8.1%) were F. Post-counselling, 24 M and 1 F had given up smoking, respectively. Out of 52 passive tobacco users, 10 (19.23%) were M and 42 (80.77%) were F. Post-counselling only 1 M and 3 F had given up smoking, respectively. 158 pts (74.88%) were unaware of the ill effects of tobacco exposure, and 53 pts (25.12%) were aware of it.

Conclusion: Tobacco use is not insignificant although it is much less among F. Unfortunately, if passive smoking is included then, F also are exposed to tobacco in a large proportion of pts. A single sitting counselling had minimal effect of the cessation of smoking indicating that it has to be a continuing process.


  P181 Category: Miscellaneous Top


Azathioprine induced aplastic crisis: The need to be cautious

Abhishek Kumar, Vivek Vasdev, GD Choudhury, Arun Hegde, Arjun MN; Army Hospital (Research & Referral) Delhi cantt

Background: Azathioprine has been the mainstay of treatment in many rheumatological illnesses and is a preferred drug due to its cost effectiveness, safety profile and efficacy. However severe life threatening bone marrow suppression in individuals with deficiency of thiopurine methyltransferase (TPMT) enzyme activity is a dreaded side effect associated with its use.

Case Summary: This 37 years old lady was a diagnosed case of systemic lupus erythematous (SLE) with diffuse proliferative lupus nephritis (Class IV), and had received induction therapy with cyclophosphamide (Euro Lupus regime) followed bymaintenance therapywith azathioprine. Eight weeks later she presented with fever, progressive fatigue, exertional dyspnea and dry cough. Preliminary investigations revealed profound pancytopenia (Hb 4.7 g/dl, Total leucocyte count 1100/cmm, Absolute neutrophil count 380/cmm, Platelet count 14000/cmm). Possibilities of SLE flare, sepsis and macrophage activation syndrome were initially considered and excluded with appropriate laboratory investigations. Patient was initially managed as a case of febrile neutropenia with cessation of azathioprine and institution of broad spectrum antibiotics with component support. The cytopenia proved refractory to therapy with Recombinant Granulocyte Colony Stimulating Factor, Erythropoietin and Eltrombopag. With no evident etiology elicited for bone marrow suppression, the patient was further assessed for TPMT mutation and was detected to be homozygous TPMT*3B (G460A) mutation with consequent low or no TPMT enzyme activity.Patient was subsequently managed supportively with broad spectrum antibiotics, antifungals and antiviral therapy and made a complete and uneventful recovery.

Conclusion: Azathioprine toxicity in a TPMT deficient patient can lead to severe morbidity or mortality, which can be easily avoided by close monitoring of the patient for evidence of myelosuppression in the initial few months of initiation of treatment. Assessment of TPMT genotype prior to commencement of therapy, although expensive can also prove to be an effective screening tool.


  P182 Category: Miscellaneous Top


A rare case of assault

Malviya Sourabh; Kela Manoj; Gupta Apoorv; Medanta Hospital, Indore, SAIMS

Panniculitis is a group of diseases whose hallmark is inflammation of the subcutaneous adipose tissue that may be challenging both for the clinician and the pathologist. Mesenteric panniculitis is a common entity, and often associated with intra-abdominal inflammatory conditions, cutaneous panniculitis is a rare entity and can be mistaken for other skin and subcutaneous lesions. We present a patient with traumatic panniculitis.

Methods & Results: A 31 year female presented with history of recurrent nodular swelling over body and extremities for 6 months on the site of assault. Her husband had beaten her up with rods 8 months before the swelling appears on the same site.

Examination: Multiple nodular lesions of varying sizes over extremities & chest and trunk

Routine investigations: unremarkable, ANA, ANCA-negative, Viral markers negative

ACE level -normal and montoux test was negative.

Skin Biopsy: Fibroadipose tissue showing chronic infiltrative infiltrate with degenerative and necrotic changes in adipocytes suggestive of panniculitis

Final Diagnosis: Tramatic Panniculitis

Treatment: She was started on steroids and azathioprine with which she showed good improvement.

Conclusion: Traumatic panniculitis is a rare condition needs to be diagnosed with good history, examination and biopsy.Surgical intervention may increase the risk of inscision related panniculitis and should be treated with steroids and immunosuppresnats and avoiding trauma.


  P183 Category: Miscellaneous Top


Hansen's Disease Presenting With Arthritis And Facial Nerve Involvement

Durgesh Srivastava, Saumya Ranjan Tripathy, Anupam Wakhlu, Puneet Kumar, Urmila Dhakad, Harikrishnan V, Rasmi Ranjan Sahoo; King George's Medical University, Lucknow, U.P

A 22 years old female, who was 7 months postpartum, presented with 4 months history of symmetrical small and large joint arthritis sparing distal interphalangeal joints. She also had numbness and tingling sensation in bilateral upper limbs and left lower limb, difficulty in closing right eye, deviation of angle of mouth to left and difficulty in clearing left foot while walking. Examination confirmed arthritis, right 7th cranial nerve LMN type palsy, left foot drop, and loss of pain and temperature sensation in bilateral upper limbs below elbow and left lower limb in sural nerve distribution. In addition, atrophy and weakness of small muscles of hands, sensory loss was present over face in the distribution of V1 and V2 divisions of bilateral 5th cranial nerves, loss of right corneal sensation. Peripheral nerve examination revealed thickened bilateral ulnar, common peroneal, right greater auricular and right supra orbital nerves. To work up distal symmetrical polyarthritis, with mononeuritis multiplex including cranial nerves, investigations revealed negative RF, ACPA, ANA, ANA, normal doppler studies and ESR was 40mm 1st hr. Once autoimmune causes were excluded, slit skin smear was ordered but was negative. Sural nerve biopsy revealed leprae bacilli in modified wade fite stain. This proved the diagnosis of Hansen's disease. This case highlights that Hansen's disease may have highly variable clinical features and atypical presentations, which are easily confused with other rheumatological diseases. This case signifies that Hansen's disease may present with cranial nerve involvement which although not uncommon, but infrequently reported.


  P184 Category: Miscellaneous Top


A rare cause of pre - gangrene - cervical rib causing thoracic outlet syndrome

Harikrishnan Velayudhan, Durgesh Srivastava, Rasmi Ranjan Sahoo, Saumya Ranjan Tripathi, Urmila Dhakad, Siddharh K Das; King George's Medical University, Lucknow

52 yr male, who is a farmer, non-smoker, presented with pain and tingling in Rt upperlimb for 2 years and bluish discoloration of all fingers of Rt hand for 2 months.Pain and tingling sensation was present over right hand and fore arm which gradually progressed and increased with shoulder abduction/extension and relieved in neutral position. He also gave history of progressively weakening handgrip and easy fatiguability of right hand.There was bluish discoloration of right hand digits; thumb, index and middle fingers. There was no history of any other systemic illness or any past illness/ surgery/ blood transfusion. Clinical examination revealed pallor, absent rt radial and ulnar artery pulsations and a weak rt brachial pulse. Blood pressure was 130/80 in both UL and 140/100 in both LL. Rt hand was cold to touch with prolonged CFT. There was no obvious supraclavicular swelling/dilated veins. subclavian bruit was present on rt side. Nervous system examination revealed wasting of muscles of thenar and hypothenar eminence with weak hand grip with reduced pain, touch and temperature in Rt hand over thenar and hypothenar areas. Possibility of thoracic outlet syndrome was considered and on examination, Adson's test, Roos test, Wright's test were positive. Routine blood investigations, inflammatory markers were normal. NCV revealed non recordable Rt median and ulnar SNAPS. Xray chest revealed B/L cervical rib. But the neuropathic symptoms and NCV findings could not be explained with the cervical rib. CT angiogaphy was done and it revealed compression of Rt subclavian artery between clavicle and cervical rib along with fibrous plaque in distal subclavian artery with thrombosis of distal Rt brachial artery, radial and ulnar arteries. Possibility of ischemic monomelic neuropathy was considered and anticoagulation was started. Patient was referred to vascular surgery department for excision of cervical rib.


  P185 Category: Miscellaneous Top


Chronic Hypertrophic Pachymeningitis, manifesting as recurrent neurological events in a 47 year old lady.

Shailee Chandak, Natasha Faria, Srinivas C, B G Dharmanand; Sakra World Hospital, Bengaluru

Idiopathic hypertrophic pachymeningitis (IHCP) is a rare inflammatory disease with diffuse involvementof the dura. The clinical presentation includes nonspecific symptoms of headache, nausea and vomiting. Other secondary manifestations includeserous otitis, retro-orbital pain, vision loss and hearing and optic neuropathy. The cause of IHCP is still speculative and relies on the exclusion of other possible causes usually with biopsy. We describe a case of Idiopathic hypertrophic pachymeningitis in 47 year old lady, presenting with recurrent neurological events. Her illnessstarted in 1996 with fever and rashes, diagnosed as cutaneous vasculitis on thebasis of a skin biopsy.She improved with steroids. In 2010, she underwent an exploratory tympanomastoidectomy for painful jaw opening. A biopsy of the granulations around stapes showed nonspecific changes, for which she was treated with antibiotics and steroids. Later in 2102, presented with acute onset diminution of vision right eye, diagnosed as Anti NMO - negative NeuromyelitisOptica. She improved with steroids and immunomodulators. Had a recurrence left eye on tapering steroid. MRI brain showed - residual empyema from previous surgery and active deymelination of optic nerve. Again in 2016, as steroids were tapered, she presented with bilateral sensorineural hearing loss. MRI Brain contrast showed diffuse thickening and enhancement of the meninges with enhancing Left 7th and 8th nerve complexes, suggestive of hypertrophic pachymeningitis. Meningeal biopsy was done and showed thick bands of fibrocollagenous tissue with focal lymphoplasmacytic infiltrate and prominent meningothelial cell proliferation with focal whorl formation compatible with Idiopathic Hypertrophic Pachymeningitis. We present here a rare cause for recurrent autoimmune neurological events, Idiopathic hypertophic pachymeningitis. Immunohistochemistry for Ig G4 is awaited. She did not have any Ig G4 disease elsewhere. This case also highlights the importance of contrast enhanced MRI in the evaluation of cranial neuropathies.


  P186 Category: Miscellaneous Top


Management of Autoimmune Ear Disease and Idiopathic Polyarthritis with Adalimumab: a Case Report

Dr. Shiva Prasad,Dr. Jagadish R Malloli, Dr. Sudhakar A; Consultant rheumatologist,Apollo BGS Hospital, Mysore, Consultant ENT surgeon, Medical Advisor, Zydus Cadila Healthcare Ltd.

Introduction: Autoimmune inner ear disease (AIED) is a rare form of sensorineural hearing loss of autoimmune origin, and responding to immunosuppressive therapy with corticosteroids or other immunosuppressants. Novel treatment approaches include intratympanic steroids and biological response modulators against TNF-α.

Case: We present a case of AIED, who concomitantly developed seronegative idiopathic polyarthritis (IPA). Treatment for IPA was initiated with methotrexate, Hydroxychloroquine and intra-articular methylprednisolone. When this regimen failed to bring adequate response, the patient was started on Adalimumab which, in addition to IPA, also showed beneficial effects on AIED.

Conclusion: Adalimumab has beneficial effect on AIED


  P187 Category: Miscellaneous Top


Cyclophosphamide in Autoimmune Rheumatic Diseases: A Systematic Review

Subramanian R1 , Vinod Ravindran2 ; JSS Medical College and Hospital, Mysore

Background: Cyclophosphamide (CP) still remains the necessary treatment in Lupus Nephritis, vasculitis and connective tissue disease related interstitial lung disease and is often the comparator with the newer agents used in these diseases. The objective of this systematic review was to evaluate the effectiveness and the incidence of toxicities of cyclophosphamide in various systemic rheumatic diseases.

Method: Literature in PubMed, Google search and Medline till July 2016 for the efficacy of CP in rheumatic diseases were identified and analyzed. Particular focus was to review CP related toxicity.

Results: Seventy studies met the inclusion criteria. Premature gonadal failure in CP treated patients ranged from 12 to 83% depending on patients' characteristics and the mode of administration. Recovery of menses or production of testosterone did not predict individual fertility. The hazard of developing ovarian failure within 10 years of cyclophosphamide initiation was one tenth in GnRH treated patients, using cox regression analysis. There was a 1.6- to 2.4-fold overall increase in malignancies, depending on the total CP dose and the time of follow-up. Skin cancer (upto 10.4-fold), lymphomas (upto 11-fold), leukemia (upto 5.7-fold) and a range of 5- to 33-fold increase in the risk of bladder carcinoma. The infection risk was high however was confounded by factors such as aggressive systemic rheumatic disease, age, mannose binding lectin deficiency and the usage of concomitant steroids.

Conclusion: Dose dependent CP induced toxicities have lead to the advent of widespread usage of alternative drugs. Usage of low dose CP regimens in the induction regimen, prophylactic usage of antibiotics and Gn RH analogues may encourage a wider acceptability of CP usage in the treatment of aggressive rheumatic diseases.


  P188 Category: Miscellaneous Top


Rheumatic Disease-Related Renal Amyloidosis: A Case Series

Ashok Kumar, Mehul Lapsiwala, Mohit Goyal, Anshul Goel, Rahul Bansal; Fortis Flt. Lt. Rajan Dhall Hospital, New Delhi

Introduction: Secondary amyloidosis associated with rheumatic diseases is rarely seen now with availability of potent therapies. In the present series we describe the clinical profile of 10 such cases seen over 7 years.

Methods: Patients with renal amyloidosis, who developed the complication as a sequel of rheumatic diseases, were recruited from our clinic. Renal histology, clinical and laboratory parameters, and clinical outcome were retrieved.

Results: There were 8 males and 2 females (mean age 45.8 years and median disease duration16 years). Five patients had ankylosing spondylitis, 4 had rheumatoid arthritis and 1 had systemic-onset JIA. Median follow-up since diagnosis of amyloidosis(biopsy-confirmed) was 20 months(range 5-48 months). Six patients received only anti-TNF therapy, 1 received 6 pulses of cyclophosphamide at monthly interval and 3 received both anti-TNF therapy and cyclophosphamide (sequentially). At the time of diagnosis of renal amyloidosis, mean serum creatininewas 2.02 mg/dL (0.6-3.9) and mean 24-hour urinary protein was 4.35 gram (1-10).At the end of available follow-up, mean 24-hour urinary protein reduced to 2.9 gram (0.3-8.9) while mean serum creatinineremained stable. At the end of follow-up, 3 patients had significant reduction in serum creatinine, 3 had worsening whereas 4 patients had no significant change in creatinine. Similarly, only 2 patients had significant reduction in proteinuria (reduction of atleast 50% or absolute value <500 mg/24 hour within first 6 months). Three patients died (heart failure-2, septicemia-1).

Conclusion: Secondary renal amyloidosis carries a grave prognosis although modern treatment has reduced the mortality to some extent.

P189

Anaemia in Rheumatoid Arthritis Patients and Correlation with Disease Activity

Siddharth Herur, Sajjan Shenoy, Damodhar Shenoy, Archith Boloor; Department of rheumatology and medicine, kasturba medical college, Mangalore

Background/Purpose: Anaemia being a common extra articular manifestation of RA also adds to the reduced quality of life, especially in the Indian female population. Hence it is important to study anaemia in active RA patients and its correlation with the disease activity.

Methods: A cross sectional study was done comparing the hemoglobin levels and type of anaemia with the disease activity. Patients diagnosed with rheumatoid arthritis (ACR-EULAR criteria 2010) were enrolled and WHO criteria for anaemia was followed.Hemoglobin levels and peripheral smear were studied in all the patients.Disease activity score (DAS28)-ESR based was calculated for all the patients.Correlation between other clinical parameters was also done.

Results: The sample size was 97. The prevalence of anaemia in the study population was calculated and stratified according to age sex and duration of disease. 84 out of 97 patients (86.6%) were found to be anaemic and 66 out of 84 (68%) had normocytic normochromic anaemia which is related to the disease activity. Disease activity was significantly high in the patients found to be anaemic (p=0.002 ). But unlike other studies there was no correlation between hemoglobin levels and duration of disease (p=0.758).

Conclusion: Highly significant correlation between hemoglobin and rheumatoid arthritis disease activity. We found no significant relation between hemoglobin and duration of disease.




 

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