단클론성 IgG 침착을 동반한 증식성 사구체 신염 1예

• Published in: Kidney research and clinical practice pp. 987 - 991
• Main Authors: 이진규, 문경철, 이지은, 김바울, 이재규, 김정화, 이광영
• Format: Journal Article
• Language: Korean
• Published: 대한신장학회 01.11.2004
• Subjects: 내과학
• ISSN: 2211-9132; 2211-9140

신장에 단클론성 면역글로빈 성분 (monoclonal immunoglobulin component)이 침착하는 질환에는 경쇄침착 질환 (light chain deposition disease, LCDD)1), AL 아밀로이드증 (AL amyloidosis)2), myeloma cast nephropathy3) 등과 같이 경쇄만 침착하는 경우와, type 1 cryglobulinemia4), 경쇄 및 중쇄 침착 질환 (light and heavy chain deposition disease, LHCDD)5), immunotactoid glomerulonephritis6), fibrillary glomerulonephritis7) 등과 같이 light chain과 heavy chain이 같이 침착하는 질환들이 있다. Proliferative glomerulonephritis with monoclonal IgG deposits is a novel disease entity characterized by ① glomerular monoclonal IgG deposits restricted to a single IgG subclass and a single light chain isotype, associated with proliferative GN; ② granular deposits by EM; ③ no clinical and laboratory evidence of cryglobulinemia. A 47-year-old male presented with edema and muscle cramps. Urinalysis revealed protein 3＋ and 5-9 RBC's/HPF. BUN/Cr were 25/1.7 mg/dL, 24 hour urine protein 5.4 g/day, and CCr 56.3 mL/min/1.73m2. Cryglobulin was negative. The histologic findings of the renal biopsy showed mesangial cell proliferation with double contour of GBM on LM, diffuse finely granular electron-dense deposit in subendothelial & mesangial area with no amyloid fibril on EM, and strong immune reactivity to IgG and kappa light chain along the glomerular basement membrane on IF. Serum and urine protein EP, IEP and immunofixation EP showed no evidence of monoclonal gammopathy. Bone marrow aspiration & biopsy were negative for plasma cell infiltration. The patient is on supportive management with an AII blocking agent KCI Citation Count: 0