Mon-022 Steroid Resistance in Childhood Nephrotic Syndrome in Maputo, Mozambique

Abstract

the ability for low viral load, untreated hepatitis B to cause proliferative glomerulonephritis (GN) or the significance of anti-Ro in absence of other biochemical, histological or clinical classical lupus manifestations. Methods: We present a case of a sixty-three-year-old female who presented with an acute on chronic renal dysfunction; she was treatment naive hepatitis B positive with low viral titre, anti-Ro antibody positive and had known IgM kappa monoclonal gammopathy with stable titres. Importantly cryoglobulins and the rest of her autoimmune panel was negative. Results: Kidney biopsy light microscopy showed proliferative GN with membranoproliferative pattern. There was moderately severe arteriosclerosis and arteriolosclerosis with few vessels showing intimal fibrinoid necrosis with hyperplasia of tunica media (onion skinning). Six out of twenty glomeruli were obsolescent and there were two fibrocellular crescents. Immunofluorescence showed 4+ granular mesangial and peripheral capillary wall reaction with IgM and kappa, 3+ granular mesangial and peripheral capillary wall reaction noted with IgG, lambda and C3 only. Electron microscopy showed variable foot process effacement and a few cryoglobulinlike cylindrical organised deposits. The patient experienced improvement in renal function with mycophenolate and prednisone, however there was debate of the use of chemotherapy agents such as rituximab or traditional myeloma therapy. We are planning to do a repeat biopsy in the future to evaluate regression of renal lesions. Conclusions: This case demonstrates the increasing evidence that many patients with proliferative glomerulonephritis do not fit neatly in to one pathophysiological box and thus treatment must be tailored to the individual patient.