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   Table of Contents - Current issue
June 2021
Volume 16 | Issue 2
Page Nos. 123-244

Online since Friday, June 25, 2021

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Coping with the Coronavirus Disease-2019 pandemic: A giant leap towards digital transformation in academic research p. 123
Latika Gupta, Salika Fakirmahmad Gadiwala
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Profile of pediatric vasculitides – Prospective hospital-based data from eastern India Highly accessed article p. 127
Arpan Saha, Sumantra Sarkar, Tanushree Mondal
Background: The pediatric vasculitides are multisystem autoimmune disorders involving the blood vessels of varying sizes. Wide geographic and ethnic variations are noted in terms of incidence and clinical features. Materials and Methods: This prospective, observational study was carried out over a period of 4 years from 2015 February to 2019 February in a tertiary care referral hospital to delineate the epidemiological pattern, clinical features, and pathological characteristics of pediatric vasculitides. All consecutive patients with vasculitis defined by the American College of Rheumatology and Chappel Hill Consensus Criteria diagnostic criteria were included in the study. They underwent relevant investigations and were treated as per the standard guidelines. Results: Of the total 110 patients with different types of vasculitis, 90% (n = 99) had primary vasculitis and the rest had secondary (10%, n = 11). Immunoglobulin A vasculitis (Henoch–Schonlein Purpura) (HSP) (n = 53, 48.19%) and Kawasaki disease (KD) (n = 35, 31.82%) were the most frequent types of the vasculitis followed by Takayasu arteritis and polyarteritis nodosa. Secondary vasculitides included those associated with systemic lupus erythematosus, juvenile idiopathic arthritis, scrub typhus infection, and drug penicillamine. Various typical and atypical features along with a definite seasonal pattern of their prevalence had been documented. Conclusion: A profile of pediatric vasculitides with a specific trend in their prevalence and seasonal pattern has been documented. HSP and KD constitute the predominant types of pediatric vasculitides. KD, although diagnosed more often than previous, HSP still outnumbers KD in Eastern India.
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Treatment adherence and disease status among children with rheumatological diseases during COVID-19 pandemic: A cross-sectional study from a tertiary care center p. 133
Sabarinath Mahadevan, Balakrishnan Navaneethan, GS Nikhila, S Sreenath, M Mahabaleshwar, S Vishnupriya, TN Tamilselvam, N Ponnien Selvan
Introduction: There is a reallocation of health-care services across the world in view of the COVID-19 pandemic. This has resulted in the disruption of routine care to children with chronic rheumatological diseases. We studied the treatment adherence and disease status of these children during the COVID-19 pandemic. Methods: We did a cross-sectional observational study of children (<16 years) with rheumatological diseases. Parents of seventy consecutive children with rheumatological diseases were interviewed through the telephone to assess the treatment adherence and disease status from April 2020 to July 2020. Results: In our study cohort, 44.3' (n = 31) had juvenile idiopathic arthritis (JIA), 52.9' (n = 37) had childhood lupus childhood-onset systemic lupus erythematosus (cSLE), 1.4' (n = 1) had juvenile scleroderma, and 1.4' (n = 1) had childhood vasculitis. About 38.6' (n = 27) had poor compliance due to reasons like difficulty in transportation (88.6') (n = 24), financial constraints (88.6') (n = 24), fear of acquiring COVID-19 by attending the study center (55.5') (n = 15), and fear of taking immunosuppressive drugs (18.5') (n = 5). Thirty percent (n = 21) respondents reported flare of symptoms. Common flare symptoms reported were joint pain (25.7') (n = 18), skin rashes (1.4') (n = 1), and oral ulcers (1.4') (n = 1). One patient with cSLE required admission for acute pancreatitis. Nearly 4.3' (n = 3) experienced influenza like illness. One patient with cSLE developed swab-positive mild COVID-19 illness. cSLE patients experienced significantly less flare symptoms compared to patients with other rheumatological illness (P = 0.03, odds ratio = 0.32, confidence interval = 0.1–0.9). Almost 98.6' (n = 68) of parents were not aware of electronic consultation portals. Conclusion: Although COVID-19 causes less symptomatic disease in children, this study highlights the indirect effects of pandemic like financial constraints and parental misconceptions on children with rheumatological diseases. Furthermore, the awareness about electronic portals in consultation needs to be increased.
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Nondigital skin ulcers in systemic sclerosis: A neglected entity p. 139
Benzeeta Pinto, Ramya Janardana, Sowmya Kaimal, B Sheba Charles, KN Sangeeta, Jasmine Mathew, Kodishala Chanakya, John Michael Raj, Vineeta Shobha
Background: Skin ulcers (SUs) are a common and difficult to manage problem in systemic sclerosis (SSc) and can be broadly classified into two types: digital ulcers (DUs) and non-DUs. Objective: The objective of this study was to analyze prevalence, associated factors, and response to therapy for non-DU. Patients and Methods: All patients with SSc who fulfilled the 2013 ACR/EULAR criteria were included in the study. SUs were divided into DU and various types of non-DU (SU on bony prominences, SU on calcinosis, SU on lower limbs, and SU with gangrene). Results: We included 146 patients with SSc (83.6' females) with a median follow-up of 20.5 months (interquartile range: 52). Eighty-four patients (57.5') had at least one episode of DU. Recurrent DUs despite vasodilators were seen in 36 (24.7') patients. Thirty-four patients (23.3') had non-DU. The most common type of non-DU was SU of lower limbs (23), followed by SU on bony prominences (19). The most common ulcer site was on the malleoli in the lower limbs and elbow in the upper limbs. Most of the ulcers healed in 3–6 months. Three patients developed osteomyelitis. The presence of non-DU was associated with longer duration of disease (P = 0.0009), occurrence of DU (P = 0.011), presence of gangrene (P < 0.001), or calcinosis (P < 0.001), and higher modified Rodnan skin score (P = 0.0013). Conclusion: Non-DUs were found in a quarter of patients with SSc. They are associated with advanced disease and vasculopathy. There is an unmet need to incorporate non-DU in clinical trial outcomes and address them in patient management guidelines.
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Granulomatosis with polyangiitis: Experience of 42 patients from a single-center, tertiary care hospital in Mumbai p. 145
Sunilkumar Rajmani Singh, Jyotsna Oak, Mahin Salim Olickal
Background: There is a paucity of data on granulomatosis with polyangiitis (GPA) from western India. The present study describes the clinical manifestations, treatment, and outcome of 42 patients from a single-center, tertiary care hospital in Mumbai. Methods: This is a retrospective observational study. From our database, patients with a diagnosis of GPA over a 10-year period (January 2010 to December 2019) were included in the study. Clinical manifestations, laboratory features, Birmingham Vasculitis Activity Score (BVAS-v3), treatment, and outcomes were analyzed. Results: Forty-two patients with GPA were identified. The median age was 48.5 years with an equal number (n = 21) of males and females. Localized, early systemic, generalized, severe, and refractory disease was seen in 4, 11, 15, 8, and 4 patients, respectively. Pulmonary manifestations (n = 27) were most common at presentation followed by renal (n = 21) and ear, nose, and throat (ENT) (n = 20). Ocular and articular disease was seen in 11 patients each. Cutaneous (n = 8) and nervous system (n = 6) involvement was less common. Unusual manifestations included suburethral mass, prostatitis, and pancreatitis. The median BVAS v3 was 14.5. Rituximab was used in 12 patients and achieved remission in 9 patients. The median duration of follow-up was 36 months. Three patients expired. Relapse of disease occurred in 17 patients. Ten patients had episodes of severe infection. No malignancy was observed. Conclusion: GPA in western India has a similar clinical profile to other parts of the country. Pulmonary, ENT, and renal involvement is common. Relapse rates are high. Rituximab is an effective therapy for severe, relapsing, or refractory disease.
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Expression of two long noncoding ribonucleic acids (growth arrest-specific 5 and H19) in rheumatoid arthritis patients: Relation to clinical characteristics and disease activity p. 152
Gehan Abdel-Fattah Hegazy, Olfat Shaker, Omnia Aly, Hanan Hassan Zaki, Mohamed R Herzalla, Ahmed Fathy Gomaa, Safaa Sayed, Maysa M Haroon
Background: Rheumatoid arthritis (RA) is a relatively common autoimmune disease in which genetic risk factors are well defined. Genetic studies are promising in exploring the long noncoding ribonucleic acids (lncRNAs): growth arrest-specific 5 (GAS5) and H19, and their possible role in the assessment of RA. Aim of the Work: The aim of the study was to describe the expression of GAS5 and H19 in serum samples of Egyptian RA patients in comparison to healthy controls, then to investigate its possible relation to RA clinical characteristics and disease activity. Patients and Methods: Levels of GAS5 and H19 expression were compared in serum samples from Egyptian RA patients (n = 100) and age- and sex-matched healthy controls (n = 100) and then correlated within the RA group to disease activity measured by the Disease Activity Score 28–erythrocyte sedimentation rate (DAS28-ESR). Results: GAS5 was downregulated (0.59 ± 0.36), while H19 was upregulated (7.41 ± 1.3) in sera of Egyptian RA patients in comparison with controls (1.07 ± 0.09 and 1.1 ± 0.09 respectively). Further analysis showed a significant negative correlation of GAS5 to DAS28-ESR (r = -0.380). However, H19 showed a positive correlation to DAS28-ESR (r = 0.487 (P < 0.05). Conclusion: GAS5 was related to less disease activity, while H19 tended to be associated with more active disease. They might be useful in RA as potential biomarkers for disease activity assessment in the following years. Still, further researches are needed to support our findings and precisely determine their potential utility in RA.
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The hindi version of mcgill pain questionnaire: A cross-cultural adaptation study in rheumatoid arthritis p. 159
Riddhi A Shroff, Twinkle Y Dabholkar
Background: Pain assessment plays a major role in guiding treatment decisions in rheumatoid arthritis (RA). The long-form McGill Pain Questionnaire (LF-MPQ) is a valid and reliable tool for multidimensional pain assessment. However, a validated Hindi version of the LF-MPQ is not available for use in India. The objective was to develop an equivalent version of the LF-MPQ in native Hindi language that is cross-culturally adapted to suit Indian patients. Methodology: After acquiring permission from the University Ethics Board, guidelines given by the Mapi Research Trust were followed and LF-MPQ was translated to culturally appropriate Hindi version. After translation, the questionnaire was subjected to testing of clinimetric properties in fifty patients with RA. Results: The Hindi version of the LF-MPQ demonstrated high levels of internal consistency (Cronbach's alpha range: 0.74–0.80) and reliability (intraclass correlation coefficient range: 0.76–0.84). The Hindi version of LF-MPQ also demonstrated moderate construct and concurrent validity when tested with Visual Analog Scale (Pearson r: 0.78) and Disease Activity Score in 28 joints (Pearson r: 0.77), respectively. Conclusion: The Hindi version of the LF-MPQ was found to be reproducible and valid for the assessment of pain in patients with RA.
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Do all patients with rheumatic Diseases have a higher risk of COVID 19? Initial results from the Karnataka Rheumatology Association COVID 19 Cohort Study (KRACC) p. 164
Vineeta Shobha, K Chanakya, Vikram Haridas, Sharath Kumar, Pramod Chebbi, Benzeeta Pinto, Vikramraj Jain, Subramaniam Ramaswamy, Shiv Prasad, Abhishek Patil, Vijay K Rao, GC Yathish, BG Dharmanand, Ramesh Jois, Ashwini Kamath, Chethana Dharmapalaiah, KN Sangeeta, Ramya Janardana, C Srinivasa, AS Harshini, Nagaraj Srinivasulu, Yogesh Preet Singh, Shweta Singhai, KM Mahendranath, S Chandrashekara
Background: Patients with autoimmune rheumatic diseases (AIRD) may be at an increased risk for COVID-19 infection and poorer outcomes when compared with the general population. We undertook this study to estimate the risk of COVID-19 infection in our AIRD population and determine parameters which contribute to its occurrence. Methods: We prospectively recruited all consecutive AIRD patients on immunosuppressive therapy from 14 specialist rheumatology centers across south Indian state of Karnataka during current COVID-19 pandemic and followed them longitudinally. Results: Among 3807 participants, the majority were women (2.9:1), mean age was 43.8 (+14.3) years, rheumatoid arthritis (52.1%), and systemic lupus erythematosus (14.8%) were the most frequent diagnosis. Twenty-three (0.6%) patients contracted SARS-CoV-2 infection. Age >60 years (P = 0.01), diabetes (P = 0.009), hypertension (P = 0.001), preexisting lung disease (P = 0.0002), current prescription of either angiotensin-converting enzyme inhibitor or angiotensin receptor blockers (P = 0.01), and higher glucocorticoids dosage (P = 0.002) were identified as potential risk factors in our cohort. The past use of cyclophosphamide (P = 0.0001) or mycophenolate mofeti (P = 0.003) or biologics (P = 0.001) also had a significant association with COVID-19 infection. Hydroxychloroquine use did not influence occurrence or outcome. The presence of underlying lung disease (relative risk - 3.08, 95% confidence interval - 1.21, 8.44, P = 0.029) was the only independent risk factor associated with the risk of COVID positivity in the multivariate analysis. Incidence rate of COVID-19 infection was similar to that of the general population (P = 0.22). Conclusions: The incidence of SARS CoV-2 infection in AIRD population is comparable to the general population. Underlying lung disease was the most important risk factor apart from older age, diabetes, hypertension, and a higher glucocorticoid dosage.
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Prescription practices, experiences, and perspectives on the usage of hydroxychloroquine among rheumatologists and other specialists p. 169
R Naveen, Alka Verma, Hussain Ahmed Raza, KG Chengappa, G S K R Naidu, Supriya Sharma, Ashish Goel, Durga Prasanna Misra, Aman Sharma, Latika Gupta, Vikas Agarwal
Background/Objectives: The use of hydroxychloroquine (HCQ) for COVID-19 has raised concerns for adverse effects. We aimed to understand the practice, perceptions, and experience of adverse drug reactions (ADRs) with HCQ use for COVID-19 and other indications. Methods: A validated e-survey with 30 questions was circulated among rheumatologists and other specialists using SurveyMonkey. Responses from rheumatologists were compared with other doctors (odds ratio [OR], median, interquartile range), and ADRs encountered based on their indications. Results: Among 410 respondents (71.2% rheumatologists, 27% academicians) with a lifetime experience of 17886 (4884–52074) patients over 12 (7–20) years, and 148 (48–349) prescription of HCQ per month, one-third (135) were managing COVID-19 with 10 (0–60) prescriptions per physician. Electrocardiograms were seldom ordered preprescription (5%), but visual scans were requested by one-thirds, especially by rheumatologists (OR-1.9). Agreement on the safety of HCQ for non-COVID indications was nearly unanimous (99%), but only two-third (64%) perceived it to be safe for COVID-19, with most (72%) being uncomfortable using HCQ with macrolides. ADRs were most often encountered after middle-age with skin pigmentation (554 [123–2063]) being the most frequent, followed by gastrointestinal intolerance (222 [42–980] per million prescriptions). Cardiac toxicity was rarely reported. ADRs other than cutaneous and visual were noted more frequently by nonrheumatologists. Rheumatologists were less likely to consider HCQ unsafe (OR-0.04) and reportedly faced a greater challenge in drug procurance (OR-2.6) during the pandemic. Conclusions: Most ADRs are rare with HCQ use in our respondent population with a large user experience. HCQ use was considered unsafe by one-thirds in the setting of COVID-19 but not outside it, lesser so by rheumatologists.
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Repurposing drugs: Lessons from rheumatology in the COVID-19 pandemic p. 179
Manesh Manoj, Prashant Bafna, Rasmi Ranjan Sahoo, Kasturi Hazarika, Anupam Wakhlu
Humanity currently faces one of its biggest challenges, created by a tiny quasi-life form, the severe acute respiratory syndrome coronavirus 2. The rapidity of spread and the enormous burden placed on public health infrastructure by the coronavirus disease 2019 (COVID-19) pandemic has forced researchers to look for quick answers for therapy. Drug repurposing is probably the quickest way to develop an effective therapy in a very short time. With additional input from artificial intelligence (AI), drug repurposing may emerge as one of the major techniques by which humanity can overcome this as well as future challenges. The field of rheumatology has been one of the biggest benefactors of drug repurposing. This article reviews the various ways drugs used in rheumatological disorders are being repurposed for possible COVID-19 treatment. An overview of other nonantiviral drugs being repurposed is also undertaken, and the role of AI in drug repurposing is touched upon.
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Role of high-density lipoprotein cholesterol in health and diseases p. 187
Brijesh Yadav, Narayan Prasad
Primary function of high-density lipoprotein cholesterol is to reverse transport the cholesterol from extrahepatic cell to liver for its metabolism and excretion. Studies have demonstrated the anti-inflammatory, anti-atherogenic, anti-oxidant, and anti-diabetic property of the high-density lipoprotein cholesterol. Although the exact mechanism still remains unelucidated, high-density lipoprotein cholesterol is a complex of lipids and proteins carrying many associated cargo molecules such as enzymes, signaling molecules, miRNA, which imparts overall functionality of high-density lipoprotein cholesterol for its beneficial health effects. However, the protein and lipids components get transform by the enzymes associated with high-density lipoprotein cholesterol, hyperglycemia, oxidative stressor, and free radicals, leading to qualitative and quantitative alterations in high-density lipoprotein cholesterol (HDLc) and diminishes its beneficial effects. In this review, we have discussed the different property of high-density lipoprotein cholesterol, its function and factors influencing HDLc property in light of anti-inflammatory activity in rheumatoid arthritis and the role of cholesterol-lowering statin drug in enhancing the beneficial effect of high-density lipoprotein cholesterol in different cell types.
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Knowledge on hydroxychloroquine prescription and attitude toward its use in COVID-19 – A survey-based study among doctors Highly accessed article p. 194
TP Afra, NA Uvais, Anuradha Bishnoi, Edavalath Sukesh, Salin Eliyas, NA Bishurul Hafi, T Muhammed Razmi
Background: The use of hydroxychloroquine (HCQ) in COVID-19 had garnered enormous media attention. There were conflicting reports as well as unscientific opinion pieces in the scientific literature also. This study was planned to assess the knowledge, attitude, and practice of health-care professionals regarding HCQ. Methods: An online survey was created with 8 knowledge-based questions (n = 2 fact-based [each scoring 1], n = 6 guideline-based [each scoring 2]), and 6 attitude-based questions, and distribution among a target population of doctors was done using Google™ forms. Results: Of 115 respondents, the majority considered HCQ a safe drug (86.1%) and were in favor of its usage in COVID-19 trials (81.7%) or health-care/contact prophylaxis (60.9%) but against its use as a mass prophylactic agent (80.9%). Contrary to the published guidelines/recommendations, 80% and 55.7% of respondents opined for routine cardiac and glucose-6-phosphate dehydrogenase evaluations before prescribing HCQ. Those who were dealing with COVID-19 patients directly (n = 41) had significantly lower knowledge score compared to the others (6.37 vs. 7.72, P = 0.007). They had significantly lesser awareness for the baseline tests recommended (97.6% vs. 77.0%, P = 0.003). A significantly higher number of these physicians opposed the use of HCQ in clinical trials (26.82% vs. 8.1%, P = 0.027) and health-care/contact prophylaxis (41.5% vs. 16.2%, P = 0.008). Conclusion: Sparse and speedily gathered information on HCQ can influence the practicing doctors, especially those involved directly in COVID-19 patient care, but less familiar with the prescription of this time-tested drug. Possibly, this has a bearing on the counseling of the patients requiring HCQ for non-COVID-19 indications and formulation of new guidelines.
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Psychological impact of coronavirus disease 2019 pandemic on patients with rheumatological disorders: A web-based cross-sectional multicentric survey p. 200
GC Yathish, Yogesh Preet Singh, Shiva Prasad, Nagaraj Srinivasulu, Sharath Kumar, R Subramanian, Pramod Chebbi, Vikramraj Jain, Naman Jain, Satish Kumar
Background: The coronavirus disease 2019 (COVID-19) pandemic has resulted in uncertainty and negative psychological outcomes for patients with autoimmune rheumatic diseases for several reasons. It is essential to understand the level of anxiety among rheumatic disease patients at the current situation of COVID-19. Objectives and Methods: A web-based cross-sectional multicenter survey was done across 5 rheumatology centers in India. The study duration was between 9th May and May 16, 2020. The questionnaire included consent, demographic details, medication history, opinion about tele rheumatology services, and anxiety score measured by Beck Anxiety Inventory. Results: The survey was sent to 2987 patients, there were 933 responses, and 581 were complete (male = 175). Complete responses were considered for data analysis. Rheumatoid arthritis was the most frequent (50.6%) diagnosis followed by other conditions. Most of the patients (43%) were on combination disease-modifying anti-rheumatic drug therapy, 12.7% of patients were on biological drugs, and 23.9% of patients were on glucocorticoids (GCs). Statistically significant higher anxiety scores were observed in females (8.79 ± 9.3), fibromyalgia patients (19.78 ± 14.8), patients on GCs (9.26 ± 8.8), and biological agents (10.65 ± 10.6). Difficulty in meeting rheumatologists and getting medications was expressed by 327 (56.2%) and 73 (29.7%) respondents, respectively. Conclusion: The findings of the study revealed that anxiety is a major concern in patients with autoimmune rheumatic diseases. Fibromyalgia patients and those who were on GC and biological drugs were found to have more anxiety levels compared to other groups of patients. There is a need to intensify the awareness and address the mental health issues.
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US7 score in the subacute phase does not correlate with outcomes in Chikungunya arthritis p. 204
Benzeeta Pinto, Anu Mohan Desai, Farha Furruqh, KN Sangeeta, B Sheba Charles, John Michael Raj, Ramya Janardana, Vineeta Shobha
Introduction: Chikungunya fever is a remerging disease and may lead to postchikungunya inflammatory joint pain. Objective: To study the clinical and ultrasound (US) features of chikungunya arthritis in the subacute phase and to determine if high disease activity on US on is associated with chronic disability. Materials and Methods: We conducted a prospective observational study of all patients who presented with subacute chikungunya arthritis over 1 year duration. Chikungunya fever was diagnosed by consistent clinical picture with specific immunoglobulin M positivity. Disease activity assessments and US-7 score were performed at baseline. Indian Health Assessment Questionnaire (HAQ) and pain on numerical rating scale were done at baseline and 1 year of follow-up. Results: We included 23 patients (18 females) with a median age of 43 years. Most (22/23) patients had polyarticular disease with negative rheumatoid factor and anticyclic citrullinated peptide. Ultrasound revealed synovitis in all and tenosynovitis in most patients. The median (interquartile range) US7 score, Disease Activity Score 28 erythrocyte sedimentation rate (DAS28-ESR) score, Indian HAQ, and pain at presentation were 15 (11), 4.4 (2), 0.92 (0.7), and 5 (6), respectively. Twenty patients were treated with a short course of glucocorticoids. One-year follow-up was available in 20 patients. The median HAQ and pain score at follow-up were 0.08 (0.21) and 1 (1), respectively . No correlation was found between US7 score, DAS28, swollen joint count, tender joint count, ESR, HAQ score, or pain at presentation with HAQ and pain at follow-up. Conclusion: Disease activity at presentation as assessed by US7 score did not correlate with disability and pain at 1 year of follow-up in patients with chikungunya arthritis.
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Pancreatitis in systemic lupus erythematosus: Clinical characterization and outcome analysis in concurrent pancreatitis and lupus p. 209
Sheba Charles, Benzeeta Pinto, Vineeta Shobha
Objective: Pancreatitis in systemic lupus erythematosus (SLE) continues is an often under-diagnosed entity with high morbidity and mortality. The objective of this case series is to study the demographics and outcomes of lupus pancreatitis and to compare with available medical literature. Materials and Methods: Records of SLE patients admitted in our hospital between January 2012 and May 2018, were examined retrospectively. Clinical and laboratory parameters of the patients who were diagnosed with SLE and pancreatitis were obtained and analyzed. Results: The prevalence of pancreatitis in our cohort was 1.33% with 77% female preponderance. All the patients presented within 3 years of lupus onset. Pancreatitis was often associated with other disease manifestations of SLE and the mean Systemic Lupus Erythematosus Disease Activity Index 2K score at presentation was 17.4 ± 7.82. None of the patients were positive for APLA antibodies. All our patients were treated with high-dose steroids and other immunosuppression as indicated for concomitant disease activity. Complications were seen in only one patient who eventually succumbed to the disease. Conclusions: Pancreatitis in SLE is associated with disease activity. High index of suspicion, early diagnosis, and institution of steroids and immunosuppressive therapy lead to a good outcome.
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Minocycline-induced antineutrophil cytoplasmic antibody-associated vasculitis in children p. 214
Sunil Kapur, Jitendra Oswal
Chronic exposure to minocycline, a commonly used anti-acne medication, results in a variety of autoimmune syndromes. Minocycline-induced vasculitis often associated with the presence of antinuclear antibody (ANA) and antineutrophil cytoplasmic antibody (ANCA) has been reported in Western literature, mainly in adults. Herein, we report a rare case of a 15-year-old male child on minocycline treatment for 1 year, who presented with fever, skin rash, and polyarthralgia for 4 months. His erythrocyte sedimentation rate (ESR) level was elevated, ANA and myeloperoxidase ANCA were positive, and skin biopsy revealed leukocytoclastic vasculitis. Upon discontinuation of minocycline and prescribing a short course of oral steroids, all his symptoms resolved and ESR normalized within 2 weeks. The child remained asymptomatic with normal acute-phase reactants at 2-month follow-up. This outcome suggested that minocycline was the main cause of ANCA-associated vasculitis.
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Antineutrophil cytoplasmic antibody-associated vasculitis in a patient with diffuse scleroderma p. 217
BN Shiva Prasad, HA Karthik Urala
Scleroderma is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The development of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in patients with scleroderma is a rare association. Renal and pulmonary complications occur more frequently than expected in scleroderma/ANCA-associated vasculitis overlap. The definitive diagnosis usually made based on autoantibody titers and biopsy of involved tissue. We present a case of diffuse scleroderma with ANCA-associated vasculitis presenting with neurological symptoms.
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IgG4 Licks base of skull, wastes tongue: An unusual presentation p. 221
Shekhar Dorle, Arun Hegde, Gunjan Dwivedi, Uday Bhanu Kovilapu, PS Mishra, Anurag Jain, Faiz Ahmed
IgG4 Related disease (IgG4 RD) is a rapidly evolving clinical entity, involving multiple organs, manifesting clinically as tumefactive lesions, histopathologically characterized by an IgG4 predominant, dense lymphoplasmacytic tissue infiltrate. It might mimic malignancy, infection as well as various other inflammatory disorders. First described in literature in 2003, in the pancreas, it has been subsequently seen to involve a wide range of organs like salivary glands, paranasal sinuses, thyroid gland, retroperitoneum, lung and kidney. Rarely, it may present as a skull base osteomyelitis (SBO). Diagnosing IgG4 RD in such patients is a herculean challenge in view of absence of systemic symptoms, difficulty in obtaining adequate tissue specimens in view of densely fibrotic cranial base lesions, as well as the close proximity of the lesions to critical neurovascular structures. The standard of care, in such cases is medical, and comprises of steroids and rituximab. Herein, we discuss one such case of IgG4 RD, who presented as multiple lower cranial nerve palsies and headache, with imaging evidence of SBO, which was diagnosed as a case of IgG4RD on histopathology, and who despite receiving the standard of care, had a poor outcome. The purpose of presenting this case is to stress upon a high index of suspicion and an aggressive management approach while treating such cases.
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Imaging of takayasu arteritis with renal infarct p. 227
Niranjan Dhanaji Kanase, Dillibabu Ethiraj, Venkatraman Indiran
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Utility of hydroxychloroquine for COVID-19: Are we hoping against hope? p. 229
KC Manish, Shailesh Advani, Latika Gupta
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Gnarled fingers and broken youth p. 231
Deeksha Elwadhi
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COVID-19 and osteoarticular tuberculosis: Challenges and perspectives from a developing nation p. 232
Karthikeyan P Iyengar, Anil Agarwal, Vijay Kumar Jain, Pranav Ish
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The untouchables p. 234
Avinash Jain, Sapan C Pandya
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Systemic lupus erythematosus presenting as autoimmune myelofibrosis and acquired hemophilia A p. 236
Mansoor C Abdulla
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Secondary antiphospholipid syndrome in an adolescent girl with sjögren syndrome p. 238
Arockia John, Dhaarani Jayaraman, Barathi Gunabooshanam, Mahesh Janarthanan
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Clinical rheumatology p. 240
Anand N Malaviya
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Rheumatology Clinics “Spondyloarthritis” p. 242
Vineeta Shobha
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Erratum: Measuring inflammatory arthritis in patients with systemic lupus erythematosus using clinical disease activity index p. 244

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