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Takayasu's arteritis (TA) is a granulomatous large vessel vasculitis more common in India. We review recently published literature in this field over the past year. Multiple reports based on genetic data and studies in peripheral blood of patients with TA suggest perturba- tion of the Th17-IL17 axis in these patients, which may be amenable to targeted therapy. Abnormal pro-atherogenic lipid profiles in TA (increased LDL-C and Apo A1, decreased HDL- C and Apo-B) may drive disease process in TA. Clinically inactive TA at diagnosis has been shown to relapse and progress angiographically on follow-up. TA seems to be associated with poorer pregnancy outcomes. Recent papers on 18 fluoro-deoxyglucose positron emis- sion tomography and contrast-enhanced magnetic resonance angiographic scoring of TA suggest the need to incorporate these techniques into clinical assessment tools to delineate the actual extent of vascular involvement in TA. Recent advances in therapeutics indicate that leflunomide may be an effective disease-modifying agent in TA, whereas a large retrospective French cohort suggests relatively safety and efficacy of biologic agents target- ing tumour necrosis factor alpha or interleukin-6 in patients with TA.

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Objective: To study the level of serum adiponectin in steroid and DMARD naïve early rheumatoid arthritis patients and its correlation with disease activity and radiographic damage. Materials and methods: Forty-three patients fulfilling ACR 2010 criteria for rheumatoid ar- thritis, with disease duration less than 2 years and 25 age, sex and BMI matched controls were recruited for the study between July 2012 to January 2013. Demographic data, tender joint count, swollen joint count, erythrocyte sedimentation rate, and rheumatoid factor status were recorded. Radiographs of hands and feet were obtained. BMI, DAS28-ESR and HAQ-DI were calculated. Serum concentration of adiponectin was measured by ELISA. Results: Among 43 early rheumatoid patients (38 females), 18 had erosive disease. There were 25 controls (21 females). Median serum adiponectin levels (14 mg/ml), range (8.5-19) were significantly higher in early RA patients compared to controls (10.2 mg/ml), range (7.9-13.5) ( p = 0.02). No difference in the adiponectin levels between erosive and nonerosive disease was noted. No correlation to duration of disease, BMI, waist hip ratio and disease activity was noted. Conclusion: In this study, while exploring the levels of adiponectin in a cohort of early RA, we observed increased serum adiponectin concentration in steroid and DMARD naοve early rheumatoid arthritis patients.

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Study design: Cross-culture validation study. Objective: To translate, culturally adapt and validate the Kannada version of Oswestry Disability Index (ODI-K), version 2.1(a). Summary of background data: The Oswestry Disability Index (ODI) has become one of the principle outcome measurement tools and frequently used evaluating disability in patients with low back pain (LBP). Until now no psychometric properties of ODI-K was available. Methods: Translation and cross-culture adaptation were performed according to the lin- guistic guideline, which included forward translation, back-translation, revision by com- mittee, and pretesting. 120 LBP patients were included to evaluate psychometric properties. Test-retest reliability was performed by administering ODI-K twice in 24 h interval. The validity was tested by comparing ODI-K with Kannada version of Roland-Morris Disability Questionnaire (RMDQ). Results : The results showed internal consistency in terms of Cronbach's a (0.99), which was excellent. Test-retest reliability was tested by using the ICC(2,1) (0.98), and it was found excellent. Constructs convergent validity was tested by comparing ODI-K with Kannada version of RMDQ which showed moderate (r = 0.54, P < 0.001) relationship. Conclusion: ODI-K is reliable and valid tool that can be used in patient oriented outcome studies for evaluating disability related to back pain among Kannada speaking population.

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Systemic lupus erythematosus and myasthenia gravis are autoimmune disorders whose association in the same patient has been rarely reported. An account of three cases of SLE and myasthenia coexisting in the same patients is being presented with a review of currently available literature. Case 1 was a 33-year-old female fulfilling 6 SLICC classification criteria for SLE. She developed diplopia, dysphagia, and hoarseness of voice. A CT thorax done for evaluation of pulmonary hypertension showed thymic enlargement and serum anti-ace- tylcholine receptor antibody was positive. The patient was treated with pyridostigmine for myasthenia gravis with clinical improvement and a subsequent 10-year uneventful follow- up. Case 2 was a 22-year-old girl with new onset proximal muscle weakness after 5 years of immunosuppressive therapy for SLE. Other potential causes of proximal weakness were ruled out. A diagnosis of myasthenia gravis was made based on anti-acetylcholine receptor antibody positivity. She later succumbed to respiratory involvement despite therapy. Case 3 was a case of myasthenia gravis diagnosed at 28 years of age based on decremental response on repetitive nerve stimulation (RNS), who presented 6 months later with pregangrenous changes in her foot. She tested positive for antiphospholipid antibody and satisfied 6 SLICC criteria for classification as SLE. She is now on treatment, well, and on long-term follow-up. In patients with SLE, development of fatigue, neurological manifestations, and muscle weakness requires a high index of suspicion to prompt a search for myasthenia in addition to myositis and drug-induced myopathy.

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There is an increased risk of infections in patients with autoimmune inflammatory rheu- matic diseases (AIRDs). The risk is more due to immune dysfunction and increased use of immunosuppressive drugs (mainly with the introduction of biologicals). Some of these are vaccine-preventable infections. In this narrative review we have appraised the recent literature on vaccinations in AIRDs along with relevant recommendations available in India.

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Presented is a case of a 55-year-old woman who was diagnosed with psoriatic arthritis at the age of 48. She is being followed up by the Internal Medicine department since 2008, when she presented with bone resorption of the distal interphalangeal joints, osteolysis at risk of proximal progression, and an active disease that caused substantial functional disability. The patient underwent an orthopedic surgery in 2012, which allowed her to recover the functional capacity of both hands. Nowadays, under methotrexate, the disease shows no signs of activity. Arthritis mutilans is the most severe and destructive form of psoriatic arthritis. The early diagnosis and the modifying antirheumatic drugs make it extremely rare.

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Objectives: The gene encoding signal transducer and activator of transcription 4 (STAT4) has been documented to be associated with rheumatoid arthritis (RA) in several populations. The aim of this study was to determine the association of the STAT4 gene polymorphism (rs7574865) with RA in the Iranian population. Methods: This study included 612 Iranian RA patients and 389 healthy control subjects. For all samples, DNA was genotyped for STAT4 G>T (rs7574865) polymorphism using the MGB TaqMan Allelic Discrimination method. Results: In our survey, the frequencies of GG, GT, and TT genotypes were 50%, 41.3%, and 8.7% in RA patients, and 51.4%, 37.5%, and 11.1% in controls (P > 0.05), respectively. STAT4 rs7574865 alleles were not associated with the disease risk in the Iranian population (P > 0.05). Conclusions: Although most studies have proposed STAT4 polymorphisms as a susceptible risk factor for RA, we did not detect any significant association between the STAT4 polymorphism and RA in our study. Population-based studies help to identify genetic determinants of disease susceptibility. Possible explanations for differences in susceptible genetic factors among populations could be different genetic predispositions and contribu- tion of gene-gene or gene-environment interactions.

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Objective: Benign joint hypermobility (BJH) is a clinical condition characterized by an increased ability of joints during passive and dynamic movements. In this study a new diagnostic criteria for generalized hypermobility in children has been proposed and compared with the Beighton criteria. Method: Two hundred children from 3 to 16 years of age were included. 100 children with BJH (according to Beighton criteria) were compared with and equal number of age and sex matched controls. The children were separately examined by two qualified pediatric rheumatologists and the Beighton score for hypermobility was calculated twice. Then examinations were performed based on the new criteria and the questionnaires were filled out. Results: Data analysis revealed significant correlation between Beighton and the new criteria in which sensitivity, specificity, positive and negative predictive values were 100%, 98%, 100%, 98%, respectively. Accuracy was 99% and balanced accuracy was 99%. The area under the ROC curve was 0.99. Conclusion: On the basis of pediatrics physiological and mechanical characteristics, it appears that the Shiari–Javadi criteria are able to detect hypermobile children more accurately. Also, the new criteria appears to be more practical, faster and easier.

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Introduction: Methotrexate (MTX) is most widely used both as monotherapy and in combi- nation therapy for the treatment of rheumatoid arthritis (RA). Combinations of different disease modifying anti-rheumatic drug/s provide additional or even have potentiating effects and therefore have become widely used. Leflunomide (LEF) alone has been seen to improve both the subjective symptoms and the objective parameters in RA. Material and methods: An open label, prospective, comparative clinical study was conducted with 100 patients, divided into two groups of 50 patients in each. Subjects in group-1 were given LEF (20 mg/day) and group-2 received a combination of MTX (initial dose of 7.5 mg/ week escalated to 25 mg/week) and hydroxychloroquine (HCQ) (200 mg twice a day). The various scores and parameters of disease activity were compared every 4 weeks for 12 weeks using Disease Activity Score (DAS28) and Clinical Disease Activity Index (CDAI) scores. Results: At 4 weeks, in group-1, DAS28 improved by 1.28 and CDAI improved by 16.82; while in group-2, DAS28 and CDAI improved by1.02 and 14.39 respectively. At 12 weeks, DAS28 and CDAI improved by 2.22 and 25.33 in group-1 and 2.35 and 26.53 in group-2 respectively. When DAS28 was compared in between groups, it was insignificant at baseline, 4 weeks, and 12 weeks with a p-value of 0.547, 0.960 and 0.182 respectively, which suggested that both groups were comparable throughout the study. The comparison of CDAI between the groups was insignificant at baseline, 4 weeks and 12 weeks with a p-value of 0.634, 0.893 and 0.333 respectively, which also suggested that disease activity in both group were comparable from baseline to 12 weeks. Conclusions: LEF was found to have equal efficacy as the combination of MTX and HCQ in reducing DAS28 and CDAI score (i.e. from severe to moderate disease activity) and so may be considered as initial therapy in RA.

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