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Mycophenolate mofetil (MMf) has recently been reported as a useful alternative immunosuppressive drug in autoimmune diseases. There is paucity of literature on its use in Takayasu's arteritis (TA). The aim of this study was to assess the safety and efficacy of MMf in Asian Indian patients with Takayasu's arteritis. Records of 21 consecutive patients with TA on treatment with oral MMF attending our centre from January 2005 to August 2008 were studied. The clinical, laboratory and angiography findings were noted and disease activity assessment was done using Indian Takayasu's arteritis activity score (ITAS) and physician's global observation score at baseline and last follow-up. Eleven patients were on steroids alone at baseline while ten patients had received azathioprine prior to administration of mycophenolate. The mean duration of follow-up on mycophenolate was 9.6 (±6.4) months. Nineteen patients (90%) received mycophenolate due to active disease, while in the other two patients, it was given to facilitate steroid tapering. Mycophenolate had to be discontinued in one patient due to skin rash. At the last visit, all the remaining 20 patients who continued mycophenolate had improvement in disease activity as evident by the drop in median ITAS [7 (range 0–19) versus 1 (range 0–7); p = 0.001]. A similar trend was noted in laboratory markers of inflammation with a reduction in mean Erythrocyte Sedimentation Rate (ESR) (68 ± 36.5 versus 43.2 ± 34 mm/first hour; p = 0.003) and mean C - Reactive protein (CRP) (31 ± 46.7 versus 17.3 ± 23.9 mg/L; p = 1.00). All patients received concomitant steroids, but there was a significant decrease in steroid dosage from 36 (±16) mg/day at baseline to 19 (±14) mg/day at last follow-up ( p < 0.001). This study is the largest series till date establishing the use of mycophenolate as a safe and effective steroid-sparing immunosuppressant in Takayasu's arteritis. [ABSTRACT FROM AUTHOR]

Human and animal model studies suggest CXCL13 is a potential biomarker in primary Sjögren’s syndrome (pSS). CXCL13 has not been studied in Indian patients with pSS. pSS cases classified by American European Consensus Group (AECG) or American college of Rheumatology(ACR) 2012 criteria, attending rheumatology clinic between July 2014 and July 2015 were included. Hospital staff and healthy, non-blood related family members of patients constituted the control group. pSS cases underwent clinical evaluation, laboratory investigations, ESSDAI and ESSPRI scoring. Unstimulated saliva was collected by the spitting method. Salivary and serum CXCL13 were quantified by indirect ELISA. CXCL13 positivity was determined using Receiver Operator Characteristic (ROC) curve. STATA13.1 (StataCorpLP,Texas,USA) software was used for statistical analysis. In this study, 45 pSS cases and 42 healthy controls were recruited. In pSS, median levels of serum CXCL13, but not salivary CXCL13 was significantly higher as compared to the corresponding levels in healthy controls (p < 0.001). Using cutoff of 43.03 pg/ml obtained by ROC, serum CXCL13 positivity was seen in 31/43(72.1%) cases and 10/34 (29.4%) controls, respectively. Serum CXCL13 levels among pSS patients on treatment, treatment naïve patients and healthy controls were statistically different. Serum CXCL13 positivity was associated with oral symptoms (p = 0.02), ocular signs (p = 0.03) and hyperglobulinemia (p = 0.01). There was no association of salivary CXCL13 level with any of the clinical variables. While serum CXCL13 was elevated in pSS, salivary CXCL13 was not. In conclusion, serum CXCL13 positivity was found to be associated with oral symptoms, ocular signs and hyperglobulinemia in pSS. [ABSTRACT FROM AUTHOR]

IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease. This disease may be associated with elevated serum and tissue IgG4 levels. Early treatment prevents fibrosis and organ damage. We retrospectively studied the clinicopathologic correlation and outcome of treatment in IgG4-RD. This single-center retrospective study was done using electronic records of patients subjected to assay of serum IgG4 levels in our laboratory by nephelometry. There were 473 patients with suspected IgG4-RD. Of them, 41 patients fulfilled comprehensive diagnostic criteria for IgG4-RD and 432 had diseases other than IgG4-RD. Clinical and histopathological data including tissue IgG4/IgG ratio, other relevant laboratory findings as well as management data of 41 patients with IgG4-RD were analyzed. There were 29 males and 12 females with mean age of 44.1 ± 2.19 years. Thirteen patients had definite, 19 had probable and 9 had possible IgG4-RD. Male predominance, multiple organ involvement and IgG4 responder Index were significantly higher in definite IgG4-RD as compared to probable and possible IgG4-RD. Serum IgG4 level was elevated in 37 patients (90.2%). Glucocorticoids were used in 35 patients (85.4%) and second-line immunosuppressive agent in 23 patients (65.7%). Of the 21 patients on follow-up, 19 (90.7%) had clinical improvement at the first follow-up visit. Nine (90%) out of the ten patients who were assessed by IgG4 responder index, also had shown improved score with treatment. Patients with IgG4-RD in our series showed favorable responses to treatment with glucocorticoids and addition of steroid sparing immunosuppressive agents (mainly mycophenolate mofetil) helped successful tapering of steroids, while maintaining the improvement. [ABSTRACT FROM AUTHOR]