P0391STUDY OF ANTI COMPLEMENT FACTOR H MEDIATED DISEASE AT A TERTIARY CARE CENTRE

Abstract Background and Aims To study the clinical profile and outcome of patients with anti complement factor H mediated disease at our centre. Method We conducted a prospective study over 24 months ( August 2016 to July 2018) to assess the clinical profile and outcome of patients with anti complement factor H mediated disease at our tertiary care centre. We had a total of 18 cases during the study period. All patients were followed up to assess their response to therapy. Results A total of 28 cases of atypical HUS were seen during the study period of which anti factor H antibody was elevated in 18 (64.2%). All patients presented with hypertension with active urinary sediments and rapidly progressive renal failure. Mean serum creatinine at presentation was 6.8 +/- 1.2 mg/dl and all patients were oliguric at presentation and required haemodialysis. Anti complement factor H antibody was elevated in all patients with a mean of 549 +/- 90 AU/ml. (normal - 0 to 100 AU/ml).Renal biopsy showed thrombotic microangiopathy in 12 patients (66.6%) while features were suggestive of C3 glomerulopathy in 6 patients (33.3%). All patients received induction with plasmapheresis and pulse steroids followed by oral steroids at 1mg / kg with IV cyclophosphamide 500mg/ m 2 monthly for 6 months . This was followed by maintenance immunosuppression with azathioprine +/- oral steroids. 10 patients (55.5%) attained complete remission. Patients with anti complement factor H mediated disease presenting with TMA had better prognosis compared to patients presenting with C3 glomerulopathy. Also patients in the paediatric age group showed good response to treatment Conclusion Our study shows that anti complement factor H mediated disease is more common in the paediatric age group with good response to plasmapheresis and immunosuppression in the form of oral steroids and cyclophosphamide. Patients presenting with TMA have better prognosis compared to C3 glomerulopathy