Korean Journal of Nephrology 2009;28(6):648-652.
A Case of Steroid-responsive C1q Nephropathy Presenting as Focal Segmental Glomerulosclerosis
Young Min Yune, M.D.1, Soo Jeong Choi, M.D.1, Na Ri Kim, M.D.1, Moo Yong Park, M.D.1, Kye Won Kwon, M.D.3, So Young Jin, M.D.2, Jin Kuk Kim, M.D.1 and Seung Duk Hwang, M.D.1
Department of Internal Medicine1 & pathology2 Soonchunhyang University, College of Medicine, Buchoen Hospital, Korea, Department of pathology3
Daejin Medical center, Bundang Jesaeng Hospital, Korea
증례 : 국소 분절성 사구체신염으로 발현한 C1q 매개성 신염의 스테로이드에 의한 관해 1예
윤영민1, 최수정1, 김나리1, 박무용1, 권계원3, 진소영2, 김진국1, 황승덕1
순천향대학교 의과대학 내과학교실1, 병리학교실2, 분당제생병원 병리과3
Abstract
C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobins and complement deposits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. Most patients with C1q nephropathy present nephrotic-range proteinuria, which has a poor response to steroid. Some patients may experience decreased renal function and progress to end stage renal disease. A 27year-old man presented with proteinuria and decreased mental state. The patient was hypertensive, with a blood pressure of 180/120 mmHg. Serum BUN/creatine was 18/1.8 mg/dL, and urinalysis revealed proteinuria (3+). Brain computed tomography showed right basal ganglial and intraventricular hemorrhage. The patient was treated with craniotomy and hematoma removal, and he also received carvedilol, losartan, nifedipine, and doxazosin for control of BP. Although his mental status recovered and blood pressure was controlled, the patient still showed subnephrotic proteinuria; therefore, renal biopsy was performed. Kidney biopsy showed segmental sclerosis in 3 out of 14 glomeruli, and mesangial C1q immunofluorescence positive staining. Electron microscopic findings revealed electron-dense deposits in the mesangium. The patient was treated with oral prednisolone, and proteinuria was alleviated after 8 weeks and remains in complete remission.
Key Words: C1q complement, Nephropathy, Focal segmental glomerulosclerosis


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