Objective: To study the clinical profile and long-term outcome of granulomatosis with polyangiitis (GPA). Methods: Records of patients with GPA attending our rheumatology clinic between April 2009 and February 2015 were retrieved. Clinical and laboratory data, details of treatment given and outcome were obtained and analysed. Remission and relapse were defined according to British Society for Rheumatology guidelines. Vasculitis damage index (VDI) was used for quantifying organ damage. Results: There were 45 patients (26 female, 19 male) with the mean age of 46 ± 12 years and median disease duration at diagnosis of 20 months. Lung, ear, musculoskeletal system, eye, nose and kidney were most frequently involved in that order. Disease was severe in 29 and limited in 16. Miscellaneous presenting features included saddle-nose deformity in 5 patients, digital gangrene in 2 and complete heart block, renal mass and renal infarction in 1 each. Tissue biopsy contributed to diagnosis in 26/30 (86%). Anti-neutrophil cytoplasmic antibody was positive in 33 (73%) patients. Prednisolone + cyclophosphamide (oral in 25, intravenous in 10) and prednisolone + rituximab were initially used for inducing remission in 35 and 4 patients, respectively. Rituximab was also used for induction in 4 patients who failed on cyclophosphamide. Mycophenolate or methotrexate were used as immunosup- pressants in limited disease. Azathioprine was used for maintenance of remission. Median follow-up was 49 months. One patient died of intractable diffuse alveolar haemorrhage and stroke within 3 months. 34 patients achieved complete remission. Sustained remission (≥2 years) occurred in 24 (median 3.5 years). Major relapse occurred in 24 (53%). Of these, 3 had a second and 2 had a third relapse. Two patients relapsed after 11 years in remission. Preferred treatment for relapse included rituximab and cyclophosphamide. Mean VDI of patients in this series was 3.02. Conclusion: GPA remits with standard treatment but relapses frequently, leading to substan- tial morbidity. Mortality is uncommon.

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Objectives: To find the prevalence of metabolic syndrome in systemic lupus erythematosus (SLE) compared to controls and to identify association of metabolic syndrome with SLE disease activity and damage. Methods: A total of 82 SLE and 82 healthy controls were studied. Metabolic syndrome was defined by National Cholesterol Education Program-Adult Treatment Panel III (NCEP ATP III), consensus definition for Asian Indian Adults and World Health Organisation (WHO) 1999 definition, and associations with lupus characteristics, disease activity, and damage were examined. Insulin resistance (IR) was estimated using the homeostasis model assessment for IR (HOMA-IR). Results: Metabolic syndrome was present in 24.39% SLE and 12.19% controls ( p < 0.04) by NCEP ATP III criteria; 29.26% SLE and 19.51% controls ( p = 0.14) by consensus definition for Asian Indians; 18.2% SLE and 7.31% controls ( p < 0.035) by WHO 1999 criteria. Hypertension and hypertriglyceridemia were more frequent in SLE than in controls. Mean body mass index, diastolic and systolic blood pressure, triglycerides, and total cholesterol were higher in SLE than in controls. HOMA-IR (median, range) was 1.31 (0.06-9.32) and 1.55 (0.01-7.92), p = 0.09 in SLE and controls, respectively. There was no association of metabolic syndrome with disease activity/damage and prednisolone use. SLE patients with metabolic syndrome had a significantly longer duration of disease compared to patients without metabolic syndrome. Conclusion: South Indian SLE patients have higher prevalence of NCEP ATP III and WHO defined metabolic syndrome compared to healthy controls. SLE patients have an altered lipid profile, but there was no IR and no association of metabolic syndrome with disease activity or damage.

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Objective: To study the profile and pattern of presentation of children with Kawasaki's disease (KD) in the state of Jammu and Kashmir. Materials and Methods: KD was diagnosed on the basis of standard diagnostic criteria. Investigations included hemogram with ESR and CRP, liver function tests, renal function tests, blood cultures, chest X-rays, electrocardiograms, and echocardiography in all patients. CT angiography was done in two patients. Results: Twenty-one (15 boys and 6 girls) children were diagnosed and or treated for the disease. Mean age at diagnosis was 3.8 years (range 0.6-14 years), and 19% of patients were above 5 years of age. Fever for more than 5 days and extreme irritability, out of proportion to the degree of fever, was a characteristic feature. Oral mucosal lesions in the form of red lips as well as fissured and cracked lips were seen in 76% and rash in 80% of cases. Lymphade- nopathy was seen in 52% of cases. Thrombocytosis was present in 77% of the patients. Three patients had platelet count more than 10 x 105/mm3. 80% patients received IVIG. Cardiac involvement in the form of myocarditis occurred in one patient, who developed cardiogenic shock and arrhythmia and died. Coronary artery aneurysm was documented in only one patient. Conclusion: KD is infrequently diagnosed in the state of Jammu and Kashmir. All clinicians, especially pediatricians need to update knowledge regarding KD, so that patients are diagnosed and treated early.

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Background: Early detection of endothelial dysfunction in patients with systemic lupus erythema- tosus (SLE) is important since they show accelerated atherosclerosis and an increased risk of cardiovascular mortality. Aim: The aim of the study was to describe the correlation of flow-mediated dilation (FMD) with common inflammatory and immunological markers in patients with SLE. An attempt was made to predict a model of FMD with the help of these markers. Material and Methods: The study included 44 treatment-naive SLE patients as per American College of Rheumatology (ACR) criteria (1982) and 44 age- and sex-matched healthy controls. Each group consists of 42 females and 2 males. FMD of the brachial artery by B-mode ultrasonography was performed in both groups to compare the FMD value. Serum hsCRP, fibrinogen, uric acid, fasting insulin, serum ferritin, total cholesterol, triglyceride, VLDL, LDL, and HDL levels were measured as markers of inflammation in SLE patients to determine whether there was any correlation with FMD. Results: There were no significant differences in age and gender between the SLE and control groups. The mean FMD was 16.85 ± 10.64 and 21.89 ± 4.6 among cases and controls, respectively. FMD was significantly less among SLE patients ( p = 0.005). The hsCRP, uric acid, fibrinogen, insulin resistance, and ferritin values were found to have significant correlations with FMD values among treatment-naive SLE patients. Multiple linear regression by the ENTER method was used to predict a model of FMD: FMD = 48.252 - 1.565 hsCRP - 0.143 fibrinogen - 0.217 uric acid + 0.001 ferritin + 7.658 IR. Conclusion: Inflammatory markers of SLE, such as hsCRP, fibrinogen, insulin resistance, and uric acid positively correlate with FMD values. FMD can be predicted from the value of these biochemi- cal parameters.

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Aims: To determine the role of IL-17 cytokine in systemic lupus erythematosus (SLE) patients and its association with clinical presentation of the disease and disease activity. Methods: 72 SLE patients and 70 healthy age and sex matched controls were included in the study. SLE disease activity was assessed in all patients with SLE disease activity index (SLEDAI-2K) scores. Plasma levels of IL-6, and IL-17 were measured by enzyme-linked immunosorbent assay and correlated their levels with clinical manifestations of the disease and SLEDAI-2K. Results: Plasma levels of IL-6 and IL-17 were significantly elevated in SLE patients than in control subjects (13.98 ± 6.95 versus 7.47 ± 1.23 pg/mL) and (19.47 ± 10.21 versus 9.93 ± 1.89 pg/mL), respectively. IL-6 and IL-17 were positively correlated with SLEDAI-2K scores (r = 0.684 at P < 0.001, r = 0.322 at P = 0.006), and lupus nephritis (r = 0.364 at P = 0.002, r = 0.474 at P < 0.001) respectively; similarly, the IL-17/IL-6 ratio was positively correlated with SLEDAI-2K (r = 0.243 at P = 0.039). Also, the level of both cytokines was positively correlated to each other during periods of disease activity (r = 0.755, P < 0.001) as well as during remission (r = 0.384, P = 0.040). Conclusion: Over-expression of IL-17 correlates with disease activity of SLE. A longitudinal study in a larger cohort of SLE patients can help validate the results.

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Aims: To explore knowledge, attitudes, and practices relating to musculoskeletal examina- tion (MSKe) in one state of India. Methods: Pediatricians from the state of Maharashtra (capital - Mumbai) were invited to complete an 11-item web-based survey. Participation implied consent and the survey was based on a similar UK study. All pediatricians from the state of Maharashtra (n = 1523) were invited to participate; 223 pediatricians responded (response rate: 14.64%) with variable training in pediatrics and experience. Results: Respondents reported similar time devoted within their consultations to manage- ment plan, history, and physical examination. Most (n = 166, 74.44%) had been taught to examine the musculoskeletal system in children (n = 82, 36.77%) or in both adults and children (n = 84, 37.67%). However, MSKe was not part of their current routine practice, despite many (n = 115, 51.57%) deeming this an important part of assessment. The majority (n = 207, 92.82%) were very confident (n = 7, 3.14%), or confident in some (n = 120, 53.81%) or most (n = 80, 35.87%) aspects of performing a structured MSKe, but were less confident with MSKe compared to other systems. Most (n = 158, 70.85%) were unaware of pGALS (Pediatric Gait Arms Legs Spine) prior to the survey but many (n = 204, 91.48%) were supportive of its inclusion within the curriculum for undergraduates and/or postgraduates, and expressed a desire to receive more information. Conclusions: Many pediatricians are not confident in MSKe and are less confident compared to other bodily systems. There is need for greater training and awareness about the importance of MSKe at both undergraduate and postgraduate level. As a simple validated clinical skill, there is considerable potential to increase teaching of pGALS and thereby ultimately potentially improve MSKe performance in clinical practice.

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The definition of osteoporosis has always been challenging. Historically the clinical defini- tion could only be based on the presence or occurrence of an osteoporotic fracture. However waiting for a fracture to occur before making a diagnosis has limitations, not least that high risk individuals cannot be identified for treatment prior to fracture. With the availability of bone density measurements, the definition moved to the use of T-scores. Whilst widely used, this approach has limitations that include low sensitivity and not taking into account other variables that influence bone strength and extra-skeletal risk factors. In view of the limitations of using T scores in isolation, there has been a move towards assessment of individualised risk that incorporates multiple risk factors (with or without bone density measurement) to help predict future fracture risk. This approach potentially allows identi- fication and treatment of individuals at high risk of fracture, the condition that needs to be treated as opposed to treating low bone density. Indeed bone density (where measured) becomes one of the many risk factors without a threshold interpretation for any given value. Numerous tools are available that have varying sensitivity, specificity, utility, applicability to, and that have been validated for any given population. Of the available tools, the World Health Organisation Fracture Risk Assessment Tool (FRAX) calculator has been extensively studied. It is available more widely, with country specific utility with and without bone density measurements, which is important in regions with scarce access to bone densitom- etry. FRAX is the only tool available that is India specific.

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