SAT-397 PAUCI-IMMUNE VASCULITIS - CLINICAL CHARACTERISTICS AND OUTCOME ANALYSIS OF 120 PATIENTS FROM A SINGLE CENTER IN INDIA

Abstract

Pauci- immune Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis causes significant morbidity and mortality. Within 1st year, infections & active vasculitis and later on cardiovascular events, malignancy and infection are causes of death. Aim: To study the outcome of pauci-immune vasculitis with renal involvement in patients at our center. To analyze whether plasmapheresis influenced outcomes in patients with pauci-immune vasculitis. Study design: It was prospective observational study of patient admitted in single tertiary center in India from 2010 to 2016 with 5 yr follow up period. Data was collected at 0 month, 3 month, 1 year, 3 year and 5 year. 3 categories were considered: 1} Renal limited disease without any other organ involvement or need for dialysis 2} Systemic disease: Renal disease with other organ involvement but no requirement of dialysis 3} Severe disease: Dialysis requiring renal disease with or without any other organ involvement. Necrotizing and crescentic glomerulonephritis consistent with diagnosis of pauci-immune renal vasculitis or anti Glomerular Basement Membrane (GBM) disease. ANCA vasculitis (anti MPO and /or anti PR3 antibody present) / anti GBM disease {anti GBM antibody present} Exclusion criteria: 1. Immune complex mediated vasculitis on histology 2. Patients without renal involvement Primary Outcomes: 1. Death: All cause & Cardiovascular 2. End stage renal disease : a. DDRD (Dialysis dependent renal failure) : Dialysis dependent for > 3 months b.NDDRD (Non Dialysis dependent renal failure) : Dialysis not required or required only for ≤ 3 month Secondary Outcomes: a. Association with anti MPO, anti PR3 and anti GBM antibodies with primary outcomes b. Role of plasmapheresis in influencing primary outcomes A total of 120 patients were evaluated. The mean age of patients was 40.3 ± 18.43 yrs, 72 % being males. 76% patients had renal limited vasculitis while 61% patients had severe vasculitis. The mean serum creatinine at baseline was 6.9 ± 4.8 mg/dl. 81% patients were put on prednisolone while 57% were given IV/oral cyclophosphamide, 15% had mycophenolate exposure and 18% had azathioprine exposure. Of 120 patients, 92 were positive for ANCA by Elisa method and Immunofixation (80 - anti-MPO antibodies and 12 - PR3 antibodies). 20 out of 39 patients tested for anti-GBM antibodies were positive. Total 11 patients were Dual Positive for anti ANCA & anti GBM antibodies. Overall 5 year patient survival for non-dialysis requiring was 95% vs 70% for dialysis requiring (p=0.0001). Renal limited vasculitis patient had overall 5 year survival of 85% which was better than those with systemic vasculitis (80%) and severe vasculitis (70%). Those who had only anti GBM had worst 5 year survival of 56% while those with dual positivity had 5 year survival of 73%, however pure ANCA positives had 80% survival with no significant difference between MPO and PR3. Plasmapheresis was beneficial in only those with NDDRF while DDRF didn’t improve. 1Vasculitis causing Dialysis dependent renal failure have poorer renal and overall survival.2Dual positive{ ANCA vasculitis and Anti GBM disease } and isolated anti-GBM disease is associated with worse outcomes as compared to those with pure ANCA vasculitis