SUN-026 MYCOPHENOLATE MOFETIL IN INDIAN PATIENTS WITH C3 GLOMERULOPATHY

Introduction: C3 glomerulopathy (C3G) is a heterogeneous disease caused by alternative complement pathway abnormalities without any standardized treatment. Immunosuppressive agent, mycophenolate mofetil (MMF), has been recently shown to be useful in treating C3G, mainly from the west. We report the clinical outcome of South-Asian (Indian) patients of C3G treated with MMF with or without steroids. Methods: The clinical and histology details of the patients of C3G treated with MMF for at least 6 months with a follow-up of at least 12 months were retrieved from the medical records of our centre over the last three years. Complete and partial remission was defined as per the KDIGO definition. Results: A total of seventeen patients with C3G were treated with MMF. The median age at presentation was 16 (IQR: 11-22) years, with five children and 12 adults. The most common mode of presentation was nephrotic syndrome (88.20%), with median serum creatinine and proteinuria of 0.80 (IQR: 0.60-1.00) mg/dL and 3.70 (IQR: 1.90-5.00) g/day at the presentation, respectively. Sixteen (94.11%) patients had microscopic hematuria. Membranoproliferative glomerulonephritis was the most common histological pattern. C3 glomerulonephritis and dense deposit disease were the reported in 9 (53%) and 8 (47%) patients, respectively. The first 10 out of 17 patients underwent serological evaluation for complement pathway abnormalities at the baseline. C3 and C4 levels were low in 12 (70.58%) and 1(5.88%) patient, respectively All the 10 (100%) patients had low alternative pathway functional assay (<28%). Five (50%) patients were positive for circulating antibodies to complement regulatory proteins. Three (30%) were positive for C3Nef, 2 were positive for anti-factor H antibody, and 1 was positive for anti-factor B antibody. Low factor B level (<85 mg/ml) and factor H level (<225 mg/ml) were seen in 1 patient, who also had positive anti-factor B antibody and C3Nef. The mean dose of MMF was 1.65 (± 0.56) g/day, and the median duration of MMF therapy was 18 months, respectively. Two-thirds (64%) of the patients responded to the treatment with complete remission in 4 (23%) and partial remission in 7 (41%) (median time: 9 months). Three patients progressed to end-stage kidney disease (ESKD) on follow-up. Of the three patients, one patient (33%) had an initial response in proteinuria to MMF but did not respond after a relapse and subsequently progressed to ESKD and another 2 (67%) patients were nonresponsive to MMF from the start of the therapy. Conclusions: Despite a small sample size and lack of a control arm, this study describes the effectiveness of MMF in treating C3G patients from South-Asia and forms a basis for future randomized trials.