POS-131 IMMUNOGLOBULIN A NEPHROPATHY AND ANCA ANTIBODIES : RARE AND SEVERE ASSOCIATION

Abstract

ImmunoglobulinA(IgA) nephropathy (IgAN), the most prevalent form of primary glomerulonephritis worldwide, is characterized by mesangial cell proliferation, matrix expansion, and mainly mesangial IgA deposition, often in conjunction with C3 and IgG and/or IgM. Its pathogenisis is enigmatic, It is reported that approximately 35-42% of IgAN patients have a history of macrohematuria before kidney biopsy. Its a benign pathology which somtimes could be associated with cresentic proliferation and more rarely by the presence of ANCA antibodies We retrospectively analyzed the epidemiologic, clinical and histological data of 27 patients with Ig A nephropathy proven by biopsy and we compared 2 groups ANCA positive and ANCA negative patients. The present study involved 27 patients, 18 men and 9 women, the average age was 27.9 years. 3 patients were ANCA positive compared to 24 ANCA negative: (2 were MPO-ANCA and 1 PR3-ANCA).ANCA positive patients were older, with more severe initial renal function, lower hemoglobin, and a higher percentage of general symptoms than the ANCA negative ones. Histologically, there was a significantly higher percentage of fibrinoid necrosis in glomeruli in ANCA-positive IgAN patients. ANCA-positive patients with IgA nephropathy had a higher percentage of crescentic glomeruli (54.5%) compared with ANCA-negative patients with crescentic IgA nephropathy (26%). The ANCA positive patients was all treated by solumedrol boli and oral corticotherapy and showed more severe prognosis in fact three patients progressed to ESKD. For ANCA-negative patients were treated with oral corticotherapy, some from the other 20 patients have been put on renin angiotensin system bloquers treatment and they showed a better renal prognosis. IgAN patients with ANCA positivity showed more severe clinical and histological features when compared with ANCA-negative IgAN patients and lower renal prognosis.