Proximal muscle weakness in children, as well as adults, can be the presenting feature of a wide range of diseases including but not limited to the idiopathic inflammatory myopathies, muscle dystrophies, metabolic, endocrine, and drug-induced myopathies. Certain diagnostic clues such as selective muscle involvement in inherited dystrophies, cramping on exertion in metabolic myopathies, diffuse muscular pain in endocrinal myopathies, and relevant drug history along with a lack of appropriate treatment response to steroids should be considered. Awareness of other possibilities and a keen eye to identify the mimics may avoid diagnostic delays and allow timely treatment. In this review, we cover some of the salient features of the various myositis mimics and the pertinent investigation findings as well as gray areas which may confound recognition of the underlying etiology. Later, we discuss key features of certain mimics while providing an overview of the differential diagnosis and clinical management for treating clinicians.

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Objectives: The objective of this study is to study the role of urinary vascular cell adhesion molecule-1 (uVCAM-1) as a marker for disease activity in lupus nephritis (LN). Methodology: This was a diagnostic study where patients with active LN were taken as cases and those without LN were taken as disease controls. uVCAM-1 was correlated with the classes of LN and standard of care markers. Results: There was a significant difference between the uVCAM-1 values in cases (59.69 [range: 0.07–13752.5]) pg/mg and controls (2.11 [range − 0.11 − 1138.5] pg/mg [P = 0.02]). Maximum levels of VCAM 1 were seen in Class 4 and Class 5 LN (P = 0.96). Although higher renal systemic lupus erythematosus disease activity index score had a higher median value of uVCAM 1, the values were not statistically significant (P = 0.2). There is a positive correlation between the uVCAM1 and anti-double-stranded DNA (anti-dsDNA) levels (r = 0.38) and a negative correlation between VCAM 1 levels and C3 (r = −0.19).The sensitivity of uVCAM1 for determining the disease activity was 65.2%, and the specificity was 75% at a cutoff value of more than 23.8 pg/mg. Conclusions: uVCAM 1, although not in isolation, but along with the other standard of care markers may be useful in assessing the disease activity.

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Spondyloarthropathies (SpA) are a group of overlapping disorders sharing certain common clinical features and genetic associations. Historically, most of the research into the pathogenesis of SpA and other types of chronic arthritis have conventionally focused on the adaptive immune system. However, recently, the pendulum has shifted in favor of innate immunity. With an aim to explore bridging concept of major histocompatibility locus (MHC)-I-opathy in context of immunopathogenesis of SpA, MEDLINE and PubMed (2000-2019) databases were searched for English language articles using keywords “MHC-I-opathy” and “spondyloarthropathy.” We reviewed papers which addressed the concept of overlap between innate and adaptive immunity and interplay between local tissue factors and innate cellular responses in the pathogenesis of SpA. The term “MHC-I-opathy” encompasses of a group of diseases which exhibit interaction between tissue-specific factors and innate immune cells. Further studies are needed for understanding of its therapeutic implication in such diseases.

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Objective: Melatonin is the major secretory product of the pineal gland and may play a role in the etiology and clinical symptoms of rheumatoid arthritis (RA). This systematic review and meta-analysis were conducted to determine the morning serum melatonin level in patients with RA compared to non-RA patients. Materials and Methods: We searched English databases (PubMed, Web of Science, Scopus, and Google Scholar) for observational studies regarding the morning serum melatonin level in RA patients from January 2000 to October 2020. The weighted mean difference (WMD) was assessed. Heterogeneity was analyzed using the Q Cochrane test and the I² measure. To assess the relationship between covariates and effect size, we performed a meta-regression analysis. Furthermore, we used the Egger test alongside the funnel plot for the assessment of publication bias. Results: The literature search revealed 214 studies, of which 7 studies met the eligibility criteria. We did not find heterogeneity (I² = 0.0%). This study showed a higher level of morning serum melatonin levels in RA patients in comparison with the controls (WMD = 5.85). In meta-regression analysis, none of the variables had a significant relationship with the efficacy of effect size. The funnel plot showed all the included studies were symmetrically distributed in the triangle area. Egger's test also showed an absence of publication bias. Conclusions: Melatonin levels were higher in RA patients than the controls, but due to the small sample size of included studies, the power of this meta-analysis was low.

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Background: Fibromyalgia is a chronic pain syndrome characterized by widespread diffuse pain and multiple tender points along with sleep disturbances, fatigue, and psychological distress. Cytokines have been proposed to play an important role in the pathogenesis of fibromyalgia. Different studies, mainly done in the Western population, have shown dysregulated cytokines in fibromyalgia patients. This study was aimed to analyze the serum cytokine patterns of interleukin-1 receptor antagonist (IL-1Ra), interleukin-8 (IL-8), and interleukin-6 (IL-6) in Indian females with fibromyalgia and its correlation with the disease severity. Materials and Methods: This single-center cross-sectional study compared the serum cytokines levels (IL-6, IL-8, and IL-1Ra) of 21 females fulfilling 2016 modification of the 2010/11 American College of Rheumatology fibromyalgia criteria and without co-existent major depressive or inflammatory diseases; with 20 age- and sex-matched controls. The outcome measures used to measure disease activity were visual analog scale pain, fibromyalgia impact questionnaire-revised, brief pain inventory pain scores, and SF-36 health survey. Statistical tests used were the Kolmogorov–Smirnov test of normality, Mann–Whitney's test, Chi-square tests, Spearman rank order, and Benjamini–Hochberg correlation. Results: Serum IL1-Ra levels were significantly lower in fibromyalgia patients, median 226.90 pg/ml (interquartile range [IQR] 476.95) than the healthy controls, median 778.80 pg/ml (IQR 721) (P < 0.05). However, there was no statistical difference in the levels of IL-8 and IL-6 between patients and the control group. The analysis for the correlation between cytokine and outcome measures failed to show any statistically significant correlation. Conclusion: Fibromyalgia patients in our study had lower serum IL-1Ra level, whereas IL-8 and IL-6 levels were normal. When compared with western studies, these contrasting results suggest a heterogeneous cytokine profile of fibromyalgia patients in different ethnic groups.

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Aim: To describe the clinical and demographic characteristics of rheumatic disease patients who were diagnosed with COVID-19 infection. To identify the risk factors associated with hospitalization of patients and find out differences if any between patients managed as in-patients and out-patients. Methods: This was a multi-center retrospective observational study done by the analysis of data collected from six tertiary care centers from April 1, 2020, to October 15, 2020. All consecutive patients with a confirmed diagnosis of rheumatic disease and with a recent history of COVID-19 illness were included in the study. Results: Our study had a total of 85 patients with 58 managed as inpatients and 27 as outpatients. Six patients needed intensive care management and two patients succumbed to the illness. The majority were females (62/85) and majority (80/85) tested positive by the Reverse transcriptase-polymerase chain reaction method. The common rheumatic disease was rheumatoid arthritis (51.7%) followed by systemic lupus erythematosus (15.2%), psoriatic arthritis (10.5%), and others. Thirty-seven patients were on steroids and 28 were on hydroxychloroquine. There was no statistical difference in the mean dose of disease-modifying drugs between the two groups. Out of hospitalized people, 84.4% were above 40 years. There was a higher prevalence of comorbidities among hospitalized patients which was statistically significant (Chi-square test, P = 0.029). There was a positive correlation between duration of stay and steroid dose (Spearman rank correlation, “r” = 0.232), which was statistically significant (P = 0.03). There was no association between stay duration and other disease-modifying agents. Conclusion: There was a positive and statistically significant correlation between steroid dose and duration of stay. The prevalence of comorbidities was higher among hospitalized patients. There was no correlation between other immunosuppressive drugs and stay duration nor was there any difference in mean dosages of these drugs between patients treated as in-patients and out-patients.

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Objectives: This study aimed to assess the rate of inactive disease in Bangladeshi patients with Takayasu arteritis (TA) treated with prednisolone and to identify the characteristics in patients that may guide in choosing induction therapy. Methods: The longitudinal study enrolled active TA patients who received 1 mg/kg/day of prednisolone. Disease activity was assessed by Indian Takayasu Activity Score - C-Reactive Protein (ITAS-CRP) and ITAS2010 at baseline and after 1, 3, and 6 months. The patients who did not achieve inactive disease or again became active during tapering of prednisolone received methotrexate. Results: Among 12 active TA patients, one patient succumbed to death after 15 days. ITAS2010 1 month after steroid showed a significant difference between remission and nonremission patients (P 0.0001). However, five out of 11 (45.45%) patients became inactive. These five patients had onset of symptoms <15 months prior to the initiation of therapy and had no history of syncope, stroke, and complications such as aortic regurgitation and hypertension. Reappearance of a pulse was noted in three out of 11 patients. The presence of carotidynia (P = 0.03) was a significant predictor for the reappearance of pulse. After 6 months, one patient (9.1%) remained inactive with prednisolone alone and other subjects received methotrexate. Conclusion: The response to prednisolone is variable in TA. The presence of carotidynia was a predictor for the reappearance of pulse. Monotherapy with prednisolone is not a good choice as an induction therapy.

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Deficiency of adenosine deaminase 2 (DADA2) is a recently identified monogenic systemic inflammatory vasculopathy with variable clinical phenotype. The majority of reported patients are in pediatric age group. In this case report, we present a case of DADA2 in an elderly female, who presented initially with multiple episodes of loose motions with bleeding per rectum and was diagnosed endoscopically as multiple rectal hemorrhagic ulcers. During her hospital stay, she had generalized tonic-clonic seizures and was detected to have a hemorrhagic stroke. The patient was managed with steroids, cyclophosphamide, and other supportive measures. She had excellent recovery with the disease in remission on low-dose steroids and mycophenolate mofetil.

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A 35-year-old Asian Indian female developed recurrent attacks of bilateral anterior uveitis with vitritis following a febrile episode. Extensive systemic investigations for a febrile episode did not yield any clue. Systemic clinical examination was also within normal limits. In this patient, we found an association of high coronavirus disease 2019 (COVID-19) total antibodies. The polymerase chain reaction for COVID-19 was negative ruling out an active COVID-19 infection. After initiation of oral steroids and immunosuppression the ocular inflammation is now under remission. This report illustrates that the presence of COVID-19 antibodies may presumably play a role in triggering the immune system, this can cause recurrent ocular inflammation in absence of other extraocular inflammation.

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Background: Disease activity in systemic lupus erythematosus (SLE) is associated with organ damage, outcome worsening, comorbidities, and mortalities in the patients. This study aimed to investigate the organ manifestation of juvenile SLE (jSLE) onset and the correlation of organ manifestation with disease activity according to the Mexican SLEs disease activity index scores. Materials and Methods: This was a cross-sectional retrospective study and was conducted at Dr. Soetomo Academic Hospital, Indonesia. The study enrolled 62 subjects with complete medical records, divided into active jSLE (39 subjects) and inactive jSLE (23 subjects). The mean age of all subjects was 11.09 years, with a male/female ratio of 1:3.9. We analyzed the patient's characteristics, laboratory results, and organ manifestations using paired sample t-test, Fischer's Exact Test, and Mann–Whitney U-Test. The correlation between organ manifestation and disease activity was analyzed using Spearman's rho correlation. The odds ratio was analyzed using Mantel-Haenszel Common Odds Ratio Estimation. Results: Malnutrition is still high in jSLE, affecting 45.16% of all subjects. Malar rash (69.23 vs. 41.67%) and bicytopenia (41.03 vs. 12.50%) were significantly higher in active jSLE than inactive. The renal manifestation was correlated with active jSLE than other organ manifestations. Renal has a 19.714-fold higher risk in active jSLE. It had been found that skin and hematological manifestations are the most typical organ manifestations. Conclusions: The renal manifestation was strongly correlated with disease activity and had the highest risk in active jSLE.

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The presence of more than one autoimmune disease in a patient can complicate not only the diagnosis but also pose a therapeutic challenge to the treating physician. Myasthenia gravis (MG) is a rare disorder which may overlap with a number of other autoimmune conditions. It is important to recognize its co-presence since the management of the accompanying autoimmune condition may need to be tailored accordingly. In this article, we describe two cases with MG overlap, one with primary Sjogren's syndrome and the other with Takayasu arteritis. Herein, we review the literature and clinical approach and management of such patients.

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Coronavirus disease 2019 (COVID-19) pandemic can cause ocular manifestations which include conjunctivitis to retinal manifestations. A 41-year-old Asian Indian male presented with unilateral blurring, glare, and pain in the left eye (LE), 22 days after COVID-19 infection. He had been administered intravenous steroids followed by oral steroids. Eye evaluation showed corrected distance visual acuity of 20/20 in both the eyes (BEs). Anterior chamber showed inflammation in the LE, a day later in the right eye. Fundus examination of the BE showed retinal pigment epithelial changes with serous elevation suggestive of multifocal serous chorioretinopathy. A month later, he had improvement in his symptoms with no cells in BE with topical steroids with resolution of serous retinopathy. Bilateral anterior uveitis possibly represents an immune-mediated trigger of his clinically quiescent psoriasis causing first-ever uveitis episode, possibly due to COVID-19. Serous chorioretinopathy could be secondary to steroids following treatment of COVID-19-related sequelae.

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Background: Buerger's disease or thromboangitis obliterans (TAO) is a segmental occlusive inflammatory condition of vessels. Assessment of disease activity in TAO is challenging. There is no dedicated prognostication score available till date for Buerger's disease. Hence, our aim was to prognosticate outcomes in patients with diagnosed Buerger's disease using Sa-MoDEI score, a novel disease extent index (DEI) derived and modified from DEI. Tak score used in Takayasu's arteritis. Patients and Methods: In this prospective observational study, patients with TAO presenting to the Department of Vascular Surgery from June 2007 to April 2009 were studied. Shinoya's criteria were used to diagnose patients with Buerger's disease. Patients were scored on the basis of vascular, laboratory, and other clinical presentations as detailed in the Sa-MoDEI pro forma. They were followed up for 12 months after baseline scoring. Amputation was considered a bad outcome. The receiver operating characteristic curve was used to delineate a specific cut-off Sa-MODEI score to define bad outcomes. Factors associated with bad outcomes were analyzed using Cox proportional hazards model. Results: There were 84 patients with Buerger's disease. All were male with a significant history of tobacco use (smoking cigarettes or “beedis” [leaf cigarettes used in south Asian nations especially in countryside], chewing tobacco, and inhaling snuff). The mean age was 39 years (standard deviation [S. D] ± 6.8). Duration of smoking was 15.35 years (S.D ± 6.2); 65 patients were in good outcome and 19 patients in bad outcome group. The optimal Sa-MoDEI score cut-off for the bad outcome was 8 which corresponded with Youden's Index. Independent factors associated with bad outcome on multivariate analysis were age of onset below 39 years (Hazard Ratio [HR] = 6.00, 95% confidence interval [CI] (1.35–26.67), P = 0.019) and Sa-MoDEI score above 8 (HR = 9.77, 95% CI (2.60–36.71), P = 0.001). Conclusions: Sa-MoDEI score can be used as a tool to predict limb salvage in Buerger's disease.

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Background: We aimed to evaluate the high-resolution ultrasonography (HRUS) findings in the hands of patients with psoriatic arthritis (PsA) and rheumatoid arthritis (RA). Materials and Methods: This was a cross-sectional study of ultrasonography (USG) of hands in patients with PsA fulfilling the Classification criteria for psoriatic arthritis (CASPAR) in comparison with cases with RA fulfilling 1987 American College of Rheumatology classification criteria. Bilateral 1^st and 2^nd metacarpophalangeal (MCP) and proximal interphalangeal joints were assessed for synovitis and bone erosions, and extensor tendons at wrist for tenosynovitis by a single observer using the GE LOGIQ BOOK XP USG machine (General Electric Medical Systems) with a high-frequency 12-MHz linear array transducer. Demographic and clinical details were extracted from the electronic database of the hospital information system. Results: A total of 62 patients (31 with RA and PsA each) were studied. Demographic and baseline features were similar, except for more male patients in PsA as compared to RA (41.9% vs. 16.1%; P = 0.02). MCP synovitis was higher in RA as compared to PsA (71% vs. 29%; P = 0.001). Multiple logistic regression analysis found that absence of MCP synovitis by HRUS (adjusted odds ratio [OR]: 5.97; P = 0.02) in spite of higher number of swollen joints (adjusted OR: 29.3; P = 0.03) favors the diagnosis of PsA as compared to RA. Conclusion: “Our finding of fewer or no MCP synovitis by HRUS in PsA as compared to RA warrants a larger, powered validation study; if reproduced, it can be an additional tool to differentiate PsA including those without overt skin lesions from seronegative RA/early undifferentiated polyarthritis.”

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Background: Kawasaki disease (KD) is a medium-vessel vasculitis having coronary predilection, usually affecting under-5 children presenting as acute febrile illness. Despite being a disease with long-term grievous outcome, only few published literature are available from India, even lesser from its eastern region. Methods: From January 2016 to December 2020, 30 case records of children with a discharge diagnosis of KD were enrolled in this study. Demographic profile, clinical manifestations, laboratory data, echocardiographic findings, and treatment done were extracted from the case records. Laboratory investigations were done at admission and repeated after 24 h of intravenous immunoglobulin administration as per the American Heart Association Guidelines 2004. Echocardiography was carried out at diagnosis, at 2 weeks, and at 6 weeks. Results: Out of 30 children diagnosed with KD, majority belong to 1–5 years of age group (72%) with male predominance. Complete KD was seen in 77% of children. The most common presentation was fever >5 days (100%) followed by oral changes in 26 (87%), conjunctivitis in 25 (83%), extremity changes in 23 (77%), and rash in 21 (70%) children. Desquamation of perineum and reactivation of bacillus Calmette–Guérin scar were seen in 10%. No children with complete KD and three children with incomplete KD developed coronary artery Abnormalities (CAA). Conclusion: Infants with incomplete KD have a higher incidence of CAA. Aggressive management results in better outcome.

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