Korean Journal of Nephrology 2009;28(6):653-657.
A Case of Pa-tient with Wegener's Granulomatosis Showing Glomerular Immune Deposition in Kidney Biopsy
Young Jin Seo, M.D.1, Yoon Jung Kim, M.D.1, Ji Mi Moon, M.D.1, Hae Yoon Choi, M.D.1, Jin-Hyun Woo, M.D.1, Seong Jae Choi, M.D.1, Young Ho Lee, M.D.1, Jong Dae Ji, M.D.1, Woon Yong Jung, M.D.2 and Gwan Gyu Song, M.D.1
Division of Rheumatology, Department of Internal Medicine1
Department of Pathology2
Korea University College of Medicine
증례 : 신장 조직 검사에서 사구체 면역침착을 보인 베게너 육아종증 1예
서영진1, 김윤정1, 문지미1, 최혜윤1, 우진현1, 최성재1, 이영호1, 지종대1, 정운용2, 송관규1
고려대학교 의과대학 류마티스 내과학교실1, 병리학교실2
Abstract
Rapidly progressive glomerulonephritis (RPGN) in Wegener's granulomatosis patients typically has been characterized by pauci-immune glomerulonephritis (PIGN). In some patients, however, significant amount of glomerular immune deposits was detected and reported that they may have poor prognosis. A 30 year-old-female visited due to the skin rash of both lower extremities, arthralgia and nasal stiffness. She had sinusitis, lung opacity, and proteinuria. Serologic PR-3 ANCA was positive and histologic findings of nasal cavity and lung also showed necrotizing vasculitis and granuloma. Thus we could diagnose Wegener's granulomatosis. However, gross hematuria developed and renal function worsened in spite of treatment with high dose prednisolone and oral cyclophosphamide. Therefore we performed a kidney biopsy. The kidney biopsy showed crescentic glomerulonephritis with Ig A deposition in the mesangium. We experienced a case of Wegener's granulomatosis patient with significant IgA deposition in glomeruli. We report this case with brief review of the literature.
Key Words: Wegener granulomatosis, Immunoglobulin A (IgA), Immune deposition
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