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  Citation statistics : Table of Contents
   2019| September  | Volume 14 | Issue 3  
    Online since October 30, 2019

 
 
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CASE BASED REVIEW
Acute pancreatitis in rheumatology practice, with emphasis on systemic lupus erythematosus: A case series and newer concepts
Praveen Pratap Jadhav, Saourabh Rajesh Desai, Janhavee Praveen Jadhav, Lata Sunil Bichile
September 2019, 14(3):229-235
DOI:10.4103/injr.injr_33_19  
Acute pancreatitis is seen in many rheumatological disorders, of which, systemic lupus erythematosus (SLE) is the most common. SLE is a multisystemic disease, affecting the gastrointestinal system in about half of the patients. Acute pancreatitis is a rare but life-threatening complication of SLE. Here, we report three intriguing cases of SLE wherein patients suffering from SLE presented with acute pancreatitis either as the initial symptom of the disease or as a flare. Another patient in this series is of dermatomyositis. Literature is reviewed, and newer concepts are discussed.
  1 561 51
LETTERS TO EDITOR
Tocilizumab-induced anaphylactic reactions in rheumatic diseases: Consideration for clinicians with monoclonal antibody infusions
Amee D Pandya, Dhaiwat Shukla, Supriya D Malhotra, Pankaj Patel, Sapan Pandya
September 2019, 14(3):251-253
DOI:10.4103/injr.injr_94_19  
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ORIGINAL ARTICLES
Platelet microparticles level in juvenile idiopathic arthritis: A pediatric population-based cross-sectional study in a tertiary care center
Naresh Kumar, K Anu Punnen, Sukesh Chandran Nair, Visalakshi Jayaseelan, T Sathish Kumar
September 2019, 14(3):182-186
DOI:10.4103/injr.injr_10_19  
Background: Platelet-derived microparticles (PMPs) are small vesicles that are released from the plasma membrane upon platelet activation, which are then involved in haemostasis, vascular health, and have recently been shown to be intimately involved in immune responses. Aims and Objectives: We aimed to evaluate the level of plasma Platelet-derived micro particles in children with JIA and to assess the relationship between PMP levels and disease activity in JIA. Materials and Methods: Children with JIA who fulfilled the International League of Associations for Rheumatology (ILAR) classification criteria for juvenile idiopathic arthritis were included. They were categorised into active disease group and inactive disease as assessed by Wallace criteria. Samples were run in Navios flow cytometer (Beckman Coulter). Platelet microparticles were identified by MPs positive for both Annexin V and CD41 antibodies. Results: Out of 26 children with JIA, 12 had active disease group and 14 had inactive disease as assessed by Wallace criteria. Mean PMP level was 83507 cells/μl and 34904 cells/μl in active and inactive disease respectively (P = 0.06). There was no significant correlation between PMP and CRP levels (P = 0.75 and r = 0 .102) or PMP and ESR levels (P = 0.56 and r = -0.186) in JIA children with active disease. Conclusion: PMP levels were significantly elevated in disease activity of JIA and could represent a new biomarker reflecting the state of cell activation in JIA. PMP role in the inflammatory processes needs to be further elucidated.
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CASE BASED REVIEW
Adenosine deaminase 2 deficiency with a novel variant of CECR1 gene mutation: Responding to tumor necrosis factor antagonist therapy
Zakiya Saleh Al Mosawi, Hiba Omar Abduljawad, Maryam Yusuf Busehail, Barrak Mahmood Al Moosawi
September 2019, 14(3):236-240
DOI:10.4103/injr.injr_36_19  
Deficiency of Adenosine deaminase 2 (DADA2) syndrome is a chronic, systemic, and inflammatory disorder, characterized by early-onset recurrent strokes, fever, livedo reticularis, and immunodeficiency. We report the case of a 4-year-old child, a product of consanguineous marriage, who presented with three episodes of hemiparesis within 1 year. She also manifested skin discoloration in the form of livedo reticularis. Workup with magnetic resonance imaging (MRI) of the brain revealed acute infarction in the right aspect of the cerebral peduncle and chronic lacunars infarct in the right thalamus with diffusion restriction. Repeated MRI after 5 months revealed diffuse loss of brain volume. The blood workup showed high inflammatory markers and significantly low adenosine deaminase 2 (ADA2) level. After being on corticosteroid and anticoagulant treatments, she suffered from a recurrent episode of cerebral infarction, after which she was commenced on tumor necrosis factor (TNF)-antagonist therapy in addition to monthly fresh plasma infusion. Thereafter, there was no cerebral insult reported for >18 months. The genetic study of the child and her parents revealed a homozygous mutation c. 336C>A, p. (His112Gln) in the CECR1 gene, and her parents were heterozygous for the same variant. This variant was not previously reported in literature. We would suggest to link this novel variant c. 336C>A, p. (His112Gln) of CECR1 gene mutation with the clinical picture, along with the positive response to TNF-antagonist therapy in the era of ADA2 deficiency syndrome.
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CASE REPORTS
Eosinophilic mastitis: A chameleon disease in rheumatologists' domain
Shruti Bajad, Rohit Bajaj, Dhaval Tanna, Muzaffar Bindroo, Kanchan Kaur, Rajiva Gupta
September 2019, 14(3):241-243
DOI:10.4103/injr.injr_39_19  
We report a rare case of eosinophilic mastitis in a young asthmatic female who presented with a breast lump and was managed conservatively with a successful outcome. Eosinophilic mastitis, characterized by eosinophilic infiltration of mammary glands, is one such entity which is frequently given a miss, and is diagnosed based on histopathological analysis. The identification of this disease is a primary step in the management of such benign disorders which can be successfully treated conservatively avoiding any surgical intervention and morbidities thereafter. Because presentation can be similar and confusing enough, we took a cue from the history of asthma, urticaria, and peripheral eosinophilia to clinch the diagnosis of eosinophilic mastitis, which was further confirmed on biopsy. To the best of our knowledge, this happens to be the youngest female patient aged 27 years who also had urticaria along with other eosinophilic manifestations, which is unreported in other cases and makes our case exceptional. She was treated with glucocorticoids and antihistaminics and is presently symptom free.
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Spondyloarthritis associated with inflammatory myositis: An unusual case report
Pradip Kumar Prajapati
September 2019, 14(3):244-246
DOI:10.4103/injr.injr_63_19  
Spondyloarthritis is an inflammatory disorder of the axial skeleton, while inflammatory myositis is an inflammatory disease affecting the striated muscles. There is no usual association of spondyloarthritis and inflammatory myositis. A 35-year-old female patient presented with proximal muscle weakness and lower back pain with a history of knee synovitis and was diagnosed to have axial spondyloarthritis with inflammatory myositis. Her radiological studies showed sacroiliitis with inflammatory edema in the thigh muscle, with laboratory investigations showing elevated levels of creatine phosphokinase and inflammatory markers and positive human leukocyte antigen B27. She responded well to treatment (prednisolone, methotrexate, analgesic, and supportive medicines) with normalization of laboratory parameters and improved clinically.
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Eyelid edema in relapsing polychondritis
Anshul Goel, Arun Gogna
September 2019, 14(3):247-249
DOI:10.4103/injr.injr_6_19  
Relapsing polychondritis (RP) is a multisystem inflammatory disease affecting all types of cartilage and proteoglycan-rich tissues with recurrent bouts of inflammation resulting in tissue damage. The pleomorphic nature, the wide spectrum of clinical features and insidious onset of the disease makes the diagnosis of RP a challenge for clinicians. Eyelid edema is a recognized manifestation of this disease frequently in association with inflammation in other parts of eyes. This case highlights eyelid edema in a patient who presented primarily with chondritis of the pinna, which subsided with steroids.
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EDITORIALS
Addressing the unmet needs in medical training for alarmingly widening demand–supply gap in rheumatic diseases: A tale of two countries
Shivani Garg, Ram Raj Singh
September 2019, 14(3):167-171
DOI:10.4103/injr.injr_100_3  
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Rheumatology teaching and training in India – From procrastination to implementation!
Rohini Handa
September 2019, 14(3):172-173
DOI:10.4103/injr.injr_110_19  
  - 355 85
Critical workforce challenges and the impact of rheumatic and musculoskeletal diseases: Urgent call for action
Sharad Lakhanpal, Akshai Lakhanpal
September 2019, 14(3):174-176
DOI:10.4103/injr.injr_600_3  
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CAR T-cells to drive away autoimmunity in lupus
Sakir Ahmed
September 2019, 14(3):177-179
DOI:10.4103/injr.injr_118_19  
  - 406 61
Platelet activation markers in juvenile idiopathic arthritis
Pandiarajan Vignesh, Surjit Singh
September 2019, 14(3):180-181
DOI:10.4103/injr.injr_400_3  
  - 234 46
LETTERS TO EDITOR
Fellowship in rheumatology
VR Joshi
September 2019, 14(3):250-250
DOI:10.4103/injr.injr_111_19  
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Allergic-dermatological symptoms and psychological variables in fibromyalgia: A preliminary study of their relationship
Juan Antonio Becerra-Garcia
September 2019, 14(3):254-256
DOI:10.4103/injr.injr_81_19  
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Ultrasound-guided suprascapular nerve block versus intra-articular steroid injection in adhesive capsulitis of shoulder: Comments on treatment options
Yeow Leng Tan, Jun Yin Lee
September 2019, 14(3):257-258
DOI:10.4103/injr.injr_116_19  
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ORIGINAL ARTICLES
Conventional synthetic disease-modifying antirheumatic drug use by race/ethnicity and factors associated with initiating biologics in Malaysian patients with rheumatoid arthritis
Suad Mohammed, Lydia Say Lee Pok, Ying Chew Tee, Fariz Yahya
September 2019, 14(3):187-193
DOI:10.4103/injr.injr_74_19  
Aims: The aim of the study was to evaluate the treatment patterns of conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), drug survival, and the factors in initiating biologic DMARDs based on race and ethnicity in rheumatoid arthritis (RA) patients. Materials and Methods: Data on RA patients, including race/ethnicity, who were attending University Malaya Medical Centre, Kuala Lumpur, Malaysia, and started on csDMARDs between January 2006 and December 2016, were collected retrospectively from the review of patients' medical records. Factors in initiating biologic DMARDs were identified. Results: A total of 369 RA patients received at least one csDMARD; 325 (88.1%) were female, and 271 (73.4%) were seropositive. Three main races were identified: Malay (28.7%), Chinese (33.1%), Indian (36.3%), and others (1.9%). Malay race patients were initiated on a csDMARD at a younger age (48.6 years, standard deviation [SD]: 12.4) due to younger age at onset compared to other races (P < 0.001). Overall, methotrexate was the most common csDMARD used, and 39 (11%) patients were on triple-combination therapy. Disease activity score 28–erythrocyte sedimentation rate improved at 3 months post-csDMARD treatment for all races (P < 0.001). Twenty-six (7%) patients received biologics, with a mean age at initiating first biologic of 49.8 (SD: 18.3) years. There were no significant differences in age at initiating first biologic between the race groups (P = 0.83). In fully adjusted models, race was not a factor in initiating biologics. Conclusion: CsDMARDs in RA were required at a younger age for a certain race due to younger age at onset. However, race does not predict the initiation of biologics and no significant difference in the use of combination csDMARDs between races.
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The impact of training through the telegram's virtual network on promoting women's knowledge and perceptions in preventing osteoporosis
Zohreh Karimiankakolaki, Maryam Khadibi, Sakineh Gerayllo, Bahareh Motaghi, Fatemeh Heidari
September 2019, 14(3):194-199
DOI:10.4103/injr.injr_70_19  
Introduction: Osteoporosis is the most common metabolic bone disease. Therefore, the present study was conducted to determine the effect of training through the Telegram network on increasing the knowledge and perceptions of women in preventing osteoporosis. Methods: This study was an educational intervention, pretest and posttest type with randomized control group. Sixty women who referred to the marginal health centers of Yazd were randomly selected and assigned to intervention and control groups. After completing the questionnaires, a package of educational messages was sent to the intervention group through the Telegram group. A month later, the questionnaires were completed again, and the data were analyzed by SPSS 18 software and Wilcoxon and Mann–Whitney test. Results: The mean age of participants in intervention and control groups was 30.46 ± 5.71 and 29.93 ± 5.02, respectively. The findings showed that the median score of knowledge (3 scores, P= 0.001) and subscales of women's beliefs (perceived sensitivity [1 score, P= 0.046], perceived severity [1 score, P= 0.001], perceived benefits [1.5 scores, P= 0.031], and cues to action [6 scores, P= 0.000]) increased significantly after intervention in the intervention group. The median score of knowledge was significantly higher than the control group (4 scores, P= 0.000). Conclusions: Use of Telegram training and virtual training spaces is effective in promoting women's knowledge and perceptions in the prevention of osteoporosis and contributes to the advancement of women's health beliefs. Therefore, the adoption of this type of training is recommended to overcome the limitations of traditional education in the prevention of osteoporosis.
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A study of serum YKL-40 and its correlation with traditional biomarkers in rheumatoid arthritis patients
Vinod Narayan, Vasanthi Pallinti, Nalini Ganesan
September 2019, 14(3):200-205
DOI:10.4103/injr.injr_44_19  
Background: This study was aimed to measure the serum levels of YKL-40 among early and late rheumatoid arthritis (RA) patients along with other disease activity measures in RA patients. Materials and Methods: It was a cross-sectional study involving 152 RA patients based on the 1987 American College of Rheumatology criteria for the diagnosis of RA and 68 age- and sex-matched healthy controls. The patient group was further subdivided into 75 early (<2 years disease duration) and 77 late (>2 years) RA patients based on the duration of the disease. Clinical examination was performed on RA patients, and traditional markers to measure the disease activity such as Disease Activity Score (DAS)-28, Visual Analog Score (VAS), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), anti-cyclic citrullinated peptide (CCP), and rheumatoid factor (RF) were assessed. Serum YKL-40 level was measured using ELISA method. All the values were expressed as median (25th–75th percentile). Results: In our study, there was a significant increase in serum YKL-40 level in RA patients (200.8 [141.24–282.7] ng/ml) compared to healthy controls (82.2 [49.01–123.78] ng/ml) with P < 0.001. There was no significant difference in serum YKL-40 levels among early and late RA patients. The traditional inflammatory markers such as ESR, CRP, and measures of disease activity such as DAS-28 and VAS were significantly increased in late RA patients than early RA (P < 0.001). Serum YKL-40 levels were not correlated with disease activity measures such as DAS-28, VAS, CRP, and ESR in RA patients. Conclusion: Serum YKL-40 level was significantly higher in RA. However there was no difference between early and late RA. It does not correlate with measures of disease activity.
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Correlation of disease activity score using erythrocyte sedimentation rate and C-reactive protein with clinical disease activity index in rheumatoid arthritis patients
Viraj Panchal, Puja Srivastava, Dhaiwat Shukla, Devang Rana, Supriya Malhotra, Sapan Pandya
September 2019, 14(3):206-210
DOI:10.4103/injr.injr_37_19  
Background: Most patients coming to government hospital are from lower socioeconomic class and do not always get investigations done like ESR or CRP. A purely clinical index like CDAI would be a useful measure in such resource poor settings. Also there is scarce data on patterns of DMARD use from our country. Objectives: To compare Disease Activity Score 28 using erythrocyte sedimentation rate (DAS28-ESR) and Disease Activity Score 28 using C-reactive protein (DAS28-CRP) with CDAI (Clinical Disease activity index) as measures of disease activity of RA and to analyze prescription patterns in RA patients. Methods: Ours was a cross-sectional study of 4 months' duration. DAS28-ESR, DAS28-CRP and CDAI were calculated. The details of DMARDs used were recorded in proformas. Correlation was done using Spearman's Correlation Coefficient Test. To assess agreement between scores Cohen's kappa value was also evaluated. P value obtained <0.05 was considered significant. Results: We evaluated a total of 104 patients most of them being females with a mean age of 45 years. The mean DAS28-ESR was 4.59 ± 1.36, mean DAS28-CRP was 3.86 ± 1.25 and mean CDAI was 16.9 ± 9.26 . The correlation coefficients of DAS28-ESR with DAS28-CRP was 0.894, DAS28-ESR with CDAI was 0.886 and DAS28-CRP with CDAI was 0.910. Methotrexate was the most prescribed drug as a mono therapy or in a combination with Hydroxychloroquine followed by Leflunomide, Sulfasalazine, and Prednisolone in that order. Conclusion: The CDAI can substitute for SDAI or DAS 28 ESR/CRP and this would be very useful in a resource poor setting. Methotrexate was the most prescribed drug in our set up as mono or combination therapy.
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Standardizing initial dilution titers of antinuclear antibodies for the screening of systemic lupus erythematosus
Joseph Sushil Rao, Vineeta Shobha, Tinku Thomas, Usha Kini
September 2019, 14(3):211-217
DOI:10.4103/injr.injr_172_18  
Background: Systemic Lupus Erythematosus (SLE), a systemic autoimmune disease, is diagnosed by correlating clinical features with a positive Antinuclear Antibody (ANA) test. Detection of ANA by Indirect Immunofluorescence (IIF) is used for screening SLE and is dependent on initial dilution titers which require population-specific standardization. With lack of exclusive commercial kits for the South Indian population, it is necessary to standardize initial screening dilution titers for ANA to distinguish SLE and other rheumatic diseases from the healthy. Methods: Newly diagnosed SLE patients between 18 and 60 years along with healthy controls from South India over 8 months (September 2015–April 2016) were selected for this prospective study. Serum samples were subjected to ANA-IIF in dilution titers of 1:40, 1:80, and 1:100 as per kit recommendations. IIF intensity and staining patterns were correlated clinically and statistically analyzed. Results: ANA positivity in dilutions of 1:40 and 1:80 was seen in 2.1% of healthy controls and negative at 1:100. About 97.9% of SLE patients were positive at 1:100 dilution; speckled pattern being the most common (52.1%), followed by homogeneous (37.4%). The patterns were best appreciated in 1:100 dilution with high significant measure of agreement of kappa between the two pathologists for both patterns and intensity at all three dilutions. Conclusion: 1:100 is the best screening dilution to distinguish SLE patterns from normal healthy individuals, and its main advantage is the delineation of various ANA patterns when positive, especially when mixed at this lower dilution.
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REVIEW ARTICLE
Recent advances in the management of antineutrophil cytoplasmic antibody-associated vasculitis
Durga Prasanna Misra, G SRSN K Naidu, Aman Sharma
September 2019, 14(3):218-228
DOI:10.4103/injr.injr_141_19  
The prognosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has remarkably improved from an almost uniformly fatal disease about three decades back, to a chronic manageable disease with survival rates in excess of 80%. Induction of remission largely relies on cyclophosphamide or rituximab-based regimens, although emerging data suggest some potential role of mycophenolate mofetil (MMF), especially in milder disease phenotypes. Recent emphasis has shifted to exploration of treatment regimens minimizing dosage and duration of corticosteroid exposure, or replacing corticosteroids altogether with agents like avacopan, in an effort to ameliorate risks due to glucocorticoids as well as minimize damage accrual. Maintenance regimens traditionally have used azathioprine, methotrexate, or less commonly, MMF. However, rituximab is emerging as a useful maintenance agent, with the advantage of single-dose administration 6 monthly. Dosing interval for rituximab maintenance is another area of active research, with emerging literature suggesting the effectiveness of a dosing schedule based on B-cell repopulation or rise in ANCA titers. Long-term considerations for patients with AAV include the risk of infections (which can be minimized with alternate-day cotrimoxazole therapy), hypogammaglobulinemia in patients on rituximab, and accrual of damage, including drug-related damage (such as diabetes and osteoporosis) and malignancies. Ongoing clinical trials attempt to decipher the role of plasma exchange, as well as delineate the exact role of newer steroid-sparing therapeutic modalities such as avacopan. Recent clinical guidelines for the management of AAV from multiple societies, including the European League against Rheumatism and the British Society for Rheumatology, provide common treatment recommendations for AAV management.
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