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   2019| December  | Volume 14 | Issue 4  
    Online since December 31, 2019

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Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces
Ankit Agrawal, Sangeetha Pabolu, Shirisha Ale, Yesenia Galan
December 2019, 14(4):317-320
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease process where the exact pathogenesis is not known. It is an independent immunological condition affecting multiple organ systems. Major manifestations include chronic urticarial vasculitis (UV) with complement deficiency and demonstration of serum C1q antibodies. We describe a case of a 60-year-old male who presented with a generalized rash and was eventually diagnosed with HUVS. Usually, HUVS and systemic lupus erythematosus (SLE) have overlapping features, and our case was unique as our patient did not have any SLE manifestations. This article emphasizes the importance of comprehensive review of systemic manifestations accompanying UV. Although rare, it raises the awareness among the physicians about its clinical presentation and diagnostic approach and also predicts the prognosis of the same.
  3,288 228 1
Experience of sarcoidosis and factors predicting relapse at a tertiary care institute in North India
Alok Nath, Zia Hashim, Ajmal Khan, Mansi Gupta, Zafar Neyaz, Durga Prasanna Misra, Ravi Mishra, Shivani Srivastava
December 2019, 14(4):265-270
Background: Sarcoidosis is a multisystem, chronic granulomatous disease of unknown etiology. The clinical course of sarcoidosis is punctuated with remission and relapses, which is unpredictable. Materials and Methods: This was a retrospective observational study in which case records of all patients with a confirmed diagnosis of sarcoidosis attending the outpatient department of pulmonary medicine department between January 2014 and December 2018 were evaluated. The diagnosis of sarcoidosis was confirmed by demonstration of noncaseating granuloma in cytopathology or histopathology along with a compatible clinical picture. Mantoux test, serum angiotensin-converting enzyme (ACE) levels, serum calcium, and 24-h urinary calcium were done. Results: Sarcoidosis was found to be more common in females. Cough, breathlessness, fever, and fatigue were the predominant symptoms. Mantoux was negative in 95.83%, raised serum ACE was seen in 65.83%, hypercalcemia in 10.83%, and hypercalciuria in 30.0% of patients, respectively. Chest radiograph revealed that 14.17% of patients presented with Stage 1, 52.50% of patients with Stage 2, 15.83% of patients with Stage 3, and 17.50% of patients with Stage 4. Mediastinal lymphadenopathy was seen in contrast-enhanced computed tomography chest in 93.33%, septal thickening with nodularity in 61.67% and fibrosis in 25.83%. Corticosteroids were started in 97.5% patients and 24.17% patients relapsed after stopping or tapering corticosteroids. Conclusion: Sarcoidosis is a common cause of bilateral hilar lymphadenopathy and interstitial lung diseases. Relapses are frequent after stopping corticosteroids, so patients should be followed up actively after stopping treatment.
  1,968 212 1
Factors predicting early discontinuation of methotrexate as a first-line treatment for rheumatoid arthritis in Italy: Results from the GISEA registry
Andreina Manfredi, Marco Sebastiani, Florenzo Iannone, Elisa Gremese, Alessandra Bortoluzzi, Ennio Favalli, Roberto Gorla, Fausto Salaffi, Enrico Fusaro, Rosario Foti, Luca Cantarini, Roberto Caporali, Alberto Cauli, Stefano Alivernini, Francesco Paolo Cantatore, Antonio Carletto, Fabrizio Conti, Salvatore D'Angelo1, Oscar Epis, Roberta Ramonda, Antonio Marchesoni, Gianfranco Ferraccioli, Giovanni Lapadula
December 2019, 14(4):271-276
Objective: Despite the well-established efficacy of methotrexate (MTX) in rheumatoid arthritis (RA), monotherapy is not sufficient in almost half of patients. The aim of this registry-based study was to detect possible predictive factors for the early failure of MTX as a first-line treatment in early RA patients. Materials and Methods: Five-hundred and ninety RA patients beginning MTX as the first-line treatment were included. Persistence on therapy was re-evaluated after 12 months. Baseline features of disease were evaluated by means of univariate Cox regression, and parameters significantly associated to the outcome were included in multivariate model. Results: One hundred and forty-nine patients (25.3%) failed MTX during the 1st year, for inefficacy in 43.6% and adverse events in 37.5% of cases, respectively. At univariate analysis, patients who discontinued or failed treatment showed lower mean age, higher prevalence of anti-citrullinated peptide antibodies (ACPAs), and higher number of tender/swollen joints. The dose of MTX was correlated with the efficacy and the tolerance of the drug. In particular, patients treated with 7.5 mg of MTX weekly showed a higher rate of discontinuation for inefficacy than adverse events, and the contrary was detected for higher doses. On multivariate analysis, age, ACPA, and number of tender joints were directly associated with MTX discontinuation or failure. Conclusions: More than 25% of RA patients treated with MTX as a first-line therapy failed treatment at 12 months. ACPA positivity, age, and number of tender joints were associated with early withdrawal of MTX in RA patients, while the dose of MTX was correlated to the efficacy and safety of the drug.
  1,631 159 -
Charlson Comorbidity Index in patients with systemic lupus erythematosus in Egypt and its relation with disease characteristics
Samah M Alian, Sahar S Khalil, Rabab S Zaghlol
December 2019, 14(4):277-282
Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder associated with various comorbid conditions. Here, we assessed the burden of comorbidity in patients with SLE in Egypt using the Charlson Comorbidity Index (CCI) and determined its association with the disease characteristics. Patients and Methods: In this retrospective study, data of patients with SLE in Egypt were collected from the medical records. This included demographic data, clinical characteristics, comorbidities, and baseline SLE disease activity index (SLEDAI) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) scores at the last visit. The CCI score was calculated for each patient. The data of deceased patients were also collected. Results: Overall, 152 patients were included in this study. The most common comorbidities excluded from the CCI were serious infections (27%) and hypertension (23%); the most common comorbidities included in the CCI, apart from the connective tissue disease, were moderate or severe renal disease (12.5%), peptic ulcer (9.9%), and diabetes mellitus (9.9%) without end-organ damage. The mean CCI score was 1.9 and was significantly correlated with the disease duration, SLEDAI, and SDI. The death rate was 7.9%; the most common cause of death was infection (50%). Death was significantly associated with a high score of SLEDAI, SDI, and CCI. The mortality rate was higher in patients with SLE along with hypertension, renal affection, hyperlipidemia, infection, and tumors (P < 0.05). Conclusion: Patients with SLE in Egypt experienced comorbidities and high CCI scores that affected their survival.
  1,512 116 -
“Spontaneous pneumomediastinum in primary Sjogren's syndrome: An uncommon manifestation”
Viswesvaran Balasubramanian, Shibdas Chakrabarti, Abhijeet Singh, Nitesh Gupta
December 2019, 14(4):312-316
Sjogren's syndrome (SS) is the second most common autoimmune disorder, after rheumatoid arthritis that affects predominantly middle-aged women. Pulmonary involvement in SS can manifest as airway abnormalities, interstitial lung disease (ILD), and lymphoproliferative disorders. Spontaneous pneumomediastinum has been reported in ILD associated with various connective tissue diseases, most frequently inflammatory myopathies (dermatomyositis or polymyositis). However, the occurrence of spontaneous pneumomediastinum in SS is uncommon. We report a rare occurrence of spontaneous pneumomediastinum in a patient with primary SS.
  1,387 105 -
The relationship between US7 ultrasound joint scoring system and disease activity score DAS28 in rheumatoid arthritis: A study in Indian population
Saurabh Maheshwari, Samar Chatterjee, Amar T Atal, DS Grewal, Vibhuti Maria
December 2019, 14(4):290-296
Introduction: US7 (7 joint ultrasound score) is an emerging ultrasound (US) joint scoring system for the assessment of disease activity in patients with Rheumatoid Arthritis (RA). Methods and Material: The study was conducted at a tertiary care center in Southern India. Sixty-two patients of RA were recruited for this cross-sectional observational study. Patients' clinical & laboratory parameters were used to calculate the disease activity score DAS28. Grayscale and Power Doppler Ultrasound (GSUS & PDUS) were performed to calculate US7 score.Statistical analysis used: Spearman's rank-order correlation, Pearson interclass correlation and ROC (receiver operating characteristic) curve analysis were performed. Results: Median DAS28 value was 4.04 with one patient in clinical remission. There was a statistically significant positive correlation (r=0.262) between synovitis by GSUS score & DAS 28 (p-value = 0.040) and synovitis by PDUS score and DAS 28 (r=0.340, p-value = 0.004). No significant correlation was found between DAS28 and tenosynovitis or erosions score. ROC curve analysis yielded the p values of 0.04 and 0.004 for synovitis by GSUS and synovitis by PDUS respectively in differentiating between the patients with low (DAS groups 1 & 2) and high disease activity (3 & 4). Conclusions: There is good correlation of the synovitis component of US7 score with DAS28. However, relationship of tenosynovitis and erosions with disease activity is questionable according to our results. An 'abbreviated US7' score with detection of only synovitis may be more suitable. This requires further validation.
  1,371 120 -
The effects of epigenetic regulation on phenotypic expressivity in Turkish patients with familial Mediterranean fever
Eser Dogan, Semra Gursoy, Giray Bozkaya, Secil Arslansoyu Camlar, Ozge Aksel Kilicarslan, Alper Soylu, Ayfer Ulgenalp, Salih Kavukcu, Ozlem Giray Bozkaya
December 2019, 14(4):297-303
Introduction: In this study, we aimed to characterize the effect of methylation on clinical diversity and gene expression levels in familial Mediterranean fever. Materials and Methods: Forty children, who were diagnosed with FMF according to the Tel-Hashomer criteria, were included in the study. The control group consisted of 32 healthy children. Demographic data, results of molecular studies, physical examination findings, number of attacks, response to treatment were recorded. To test the possibility that methylation is responsible for different phenotypic reflections of mutations, we classified FMF patients with respect to MEFV mutations and studied the differences of MEFV mRNA, pyrin and methylation levels between different subgroups. Results: MEFV mRNA expression levels were significantly lower in FMF patients, compared to control subjects (P = 0.049). Consistent with this observation, pyrin levels were significantly lower in FMF patients (P = 0.037). In addition, we found that MEFV methylation level was higher in FMF patients compared to control subjects (P = 0.376). Conclusions: There was no evidence that differences in methylation levels could be responsible for variable effects of different mutations and variable clinics in the MEFV gene. Further studies on larger patient groups are necessary to identify the effects of different factors.
  1,347 120 -
The increased erythrocyte membrane n-6/n-3 fatty acids ratio and inflammatory markers in patients with psoriasis
Serap Ozer Yaman, Asım Orem, Fulya Balaban Yucesan, Savas Yayli, Sadık Ozturk, Sevgi Bahadir
December 2019, 14(4):283-289
Background: Psoriasis is a common chronic, recurrent inflammatory skin disorder. Omega-3 fatty acids exhibit low inflammatory effects, while n-6 fatty acids display high inflammatory effects. The purpose of this preliminary study was to determine erythrocyte membrane n-6/n-3 fatty acid ratio values and their relationship with plasma inflammatory mediators in patients with psoriasis. Methods: The study groups consisted of thirty patients with psoriasis and 36 healthy controls. Analyses of fatty acids in the erythrocyte membrane were performed using gas chromatography and a flame ionization detector. Serum sample inflammation parameters (C-reactive protein [CRP]), erythrocyte sedimentation rate (ESR), and interleukin-6 (IL-6) were also analyzed. Results: The erythrocyte membrane n-6/n-3 fatty acid ratio was significantly higher in the psoriatic group compared to the healthy control group (P = 0.004). CRP, ESR, and IL-6 levels were also higher (P < 0.05) in the psoriatic group. The n-6/n-3 ratio was positively correlated with CRP and IL-6 levels in the psoriatic group (rho = 0.687, P= 0.0001 and rho = 0.542, P= 0.002, respectively). ROC curve analyses of CRP, ESR, IL6, and n6/n3 ratio values in patients with psoriasis performed similarly. Conclusion: This preliminary study indicates that erythrocyte membrane n6/n3 fatty acid ratio was increased and showed correlations with plasma CRP and IL-6 in patients with psoriasis. Receiver operating characteristic analysis indicates that the n6/n3 ratio (area underthe curve = 0.699) may be a suitable inflammatory marker for further exploration in psoriasis. Erythrocyte membrane n-6/n-3 fatty acid ratio may be used as one of the pro-inflammatory markers in psoriasis.
  1,237 122 -
Sustained remission in large-Vessel vasculitis: Do they ever burn out?
Sakir Ahmed, Padmanabha Shenoy, Debashish Danda, Ramnath Misra
December 2019, 14(4):304-311
Drug-free remission is the ideal end point for any chronic disease. Although there are data on drug-free remission in rheumatoid arthritis and lupus, such data are limited for most vasculitis. Notably, there is less evidence for disease-modifying agents in large-vessel vasculitis (LVV). Thus, we explored the literature about sustained remission in LVV. MEDLINE and SCOPUS were searched for outcomes in LVV, and the results were manually screened for studies with at least 1-year follow-up. Isolated polymyalgia rheumatica was not included. In giant cell arteritis (GCA), histological and clinical remissions were discordant. Histology could not predict relapse rate. Various imaging techniques exhibit vessel wall inflammation in clinically quiescent disease. Relapse rate seems to correlate with the rate of steroid reduction. Relapse was rare when on higher steroid doses. Emerging evidence suggests that tocilizumab and methotrexate may prevent relapse. In Takayasu arteritis (TA), histology specimens are difficult to obtain. Remission on imaging does not mirror clinical remission. While magnetic resonance imaging and positron emission tomography are sensitive tools, these cannot differentiate smouldering disease from vascular repair. The best predictor of relapse is the extent of disease. Approximately half of the TA patients relapsed by 5 years. In patients undergoing intravascular procedures, restenosis occurred in around a third. Even for patients on anti- Tumour necrosis factor necrosis factor, sustained remission was in 20% only. LVV seems to be steroid dependent, and the efficacy of various steroid-sparing agents cannot be established unless the natural history of the disease is known. Both TA and GCA can have grumbling courses with relapse rates increasing over time.
  1,164 107 -
Single-organ sarcoidosis involving hepatic, lacrimal, and Cardiac
BN Shiva Prasad, Ravi Puranik, Venugopal Anandaswamy, P Jayakumar
December 2019, 14(4):321-324
Sarcoidosis is a multisystem idiopathic granulomatous disease. Lung is the most common organ involved but can involve any organ. The skin, eyes, joints, and heart are affected in a significant fraction of patients. Extrapulmonary sarcoidosis may be the major manifestation of the disease. Extrapulmonary disease may manifest before, concurrent with, or after the development of pulmonary disease or as isolated independent disease. We present three cases of isolated organ sarcoidosis involving the liver, lacrimal, and heart. Isolated organ involvement is difficult to diagnose and the treatment of Isolated organ is not defined.
  1,152 116 -
Pulmonary sarcoidosis – Predictors of relapse: Are we there yet?
Vineeta Shobha
December 2019, 14(4):259-260
  1,108 141 -
Lipidomics in psoriatic disease: The new kid on the omics block
Ashish J Mathew, Vinod Chandran
December 2019, 14(4):261-262
  1,008 133 -
Postmalignancy sarcoid-like reaction
Shiva Prasad, Nairutya Shivathirthan, Keyur Yogesh Patel
December 2019, 14(4):327-329
  943 80 -
Bone mineral density is decreased in patients with systemic sclerosis and correlates with serum intact parathormone levels: A cross-sectional study
Mahmood Dhahir Al-Mendalawi
December 2019, 14(4):325-325
  919 90 -
Adiponectin and rheumatoid arthritis
Beuy Joob, Viroj Wiwanitkit
December 2019, 14(4):330-330
  867 75 -
Response to comments on “bone mineral density is decreased in patients with systemic sclerosis and correlates with serum intact parathormone levels: A cross-sectional study”
Shefali Sharma, Aadhaar Dhooria, Tulika Singh
December 2019, 14(4):326-326
  829 65 -
Rheumatology in India: Yesterday, today, and tomorrow?
Robert J Moots
December 2019, 14(4):263-264
  758 127 -