Thin Glomerular Basement Membrane Disease를 동반한 베게너육아종증 1예

Abstract

Wegener`s granulomatosis is a necrotizing, granulomatous vasculitis that involves multiple organs including the upper and lower respiratory tract and the kidney. The kidney initially exhibits focal necrotizing glomerulonephritis, which progresses to crescentic glomerulonephritis in Wegener`s granulomatosis. We experienced a case of Wegener`s granulmatosis which was associated with a thin glomerular basement membrane disease. The patient suffered from nasal stuffiness, recurrent serous otitis media, and tinnitus. Despite antibiotic therapy and ventral tube insertion, symptoms did not improve and hearing difficulty was aggravated. Ulcerative, necrotizing granulomatous inflammations with multinucleated giant cells were seen on nasal biopsy. She had recurrent microscopic hematuria and the renal biopsy findings by light and immunofluorescent microscopy did not reveal any abnormalities but diffuse thinning of the glomerular basement membrane (226nm) was observed by electronmicroscopy. With the above clinical findings and biopsy results, we diagnosed Wegener`s granulmatosis with thin glomerular basement membrane disease. Thin glomerular basement membrane disease, also called benign recurrent hematuria, is characterized by diffuse thinning of the glomerular basement membrane and hematuria. Weekly low-dose methotrexate together with prednisone was used as treatment regimen because nonglomerular microscopic hematuria may be the first sign of cyclophosphamide-induced renal toxicity. With the above combination therapy, she felt well-being sense and her hearing difficulty was also much improved. She has been treated as an outpatient with glucocorticoid.