MON-004 PROFILE AND OUTCOMES OF PRIMARY NEPHROTIC SYNDROME

Abstract

Primary nephrotic syndrome encompasses a wide range of histopathological diagnoses with varied incidence, presentations, treatment options, response and outcomes based on geographical distribution, age, sex and the histopathological diagnosis. Our aim was to study the outcomes of primary nephrotic syndrome in our patient population. The study was conducted in two arms: retrospective arm (From 1st January 2013 to 28th February 2016) and prospective arm (1st March 2016 to 28th February 2017). All biopsy proven cases of primary nephrotic syndrome that were on regular follow up in the department of nephrology in our institute for a minimum of 6 months were included. Primary outcomes (treatment responses) and secondary outcomes (complications due to treatment or disease per se) were studied. Total 196 patients (140 retrospective and 56 prospective) were included. The mean age of patients was 33.6±17.7 years with male to female of 2:1. The most common etiological diagnosis was idiopathic membranous nephropathy (MN) in 36.7% followed by minimal change disease (MCD) in 28.1%, focal segmental glomerulosclerosis (FSGS) in 20.9%, membranoproliferative glomerulonephritis (MPGN) in 9.7%, IgM nephropathy in 2.6% and IgA nephropathy in 2.0%. Oral prednisolone was used as the primary treatment in all cases except patients of MN where oral prednisolone along with either oral cyclophosphamide or tacrolimus was given. Secondary treatment was given to patients who showed no response, partial response or relapsed as well as steroid dependent patients. Secondary and in some cases tertiary treatment was given in the form of oral prednisolone combined with oral cyclophosphamide or tacrolimus or mycophenolate mofetil or rituximab intravenous infusion. The cumulative rate of complete remission was highest in MCD (93%), followed by FSGS (85%), MN (83%), IgM nephropathy (80%), primary MPGN (37%) and IgA nephropathy (25%). The rate of relapse was highest in IgM nephropathy (60%) followed by MCD (55%), FSGS (17%), and MN (11%). The rate of steroid dependence was 25% in MCD and 50% in IgM nephropathy. The rate of steroid resistance was 6% in MCD and 16% in FSGS. The rate of complete remission with oral prednisolone alone was 81% in MCD and 65% in FSGS. The rate of complete remission with oral prednisolone combined with oral cyclophosphamide was 83% in MCD and 57% with MN. The complete remission rate on oral prednisolone combined with tacrolimus was 85% in MN, 81% in MCD and 78% in FSGS. Secondary outcomes included complications such as pneumonia in 19 cases, urinary tract infection in 12 cases, diabetes mellitus due to tacrolimus in 11 cases and neutropenia due to cyclophosphamide in 11 cases. Five cases of MCD had spontaneous bacterial peritonitis. Two cases of deep vein thrombosis and one case of renal vein thrombosis were seen in membranous nephropathy. The profile of nephrotic syndrome and the therapeutic options used have shown a favorable response to outcomes of primary nephrotic syndrome with the exception of MPGN and IgA nephropathy.