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Original Article
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Korean J Med. 2009;76(1):154-157.
- 전신성 경화증 환자에서 발생한 ANCA 양성의 급성 진행성 사구체신염 1예
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권정석, 신영국, 곽진호, 박성훈, 김성규, 최정윤
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- ANCA-related crescentic glomerulonephritis in a patient with systemic sclerosis
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Jung Seok Kwon, Young Kook Shin, Jin Ho Kwak, Sung-Hoon Park, Seong-Kyu Kim, Jung-Yoon Choe
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- Abstract
- The renal manifestations of systemic sclerosis include proteinuria, hypertension, azotemia, and renal crisis. Two types of scleroderma
renal crisis (SRC) are recognized. Typical SRC is a syndrome consisting of acute-onset malignant hypertension accompanied
by rapidly progressive renal failure, hypertensive retinopathy, and elevated plasma renin activity. The other type is normotensive
renal failure, which is generally accompanied by antineutrophil cytoplasmic autoantibody (ANCA)-positive crescentic
glomerulonephritis. A 51-year-old woman with scleroderma without marked dermatological change developed ANCA-related renal
failure. She had neither malignant hypertension nor an elevated plasma rennin concentration. Renal biopsy showed crescentic
glomerulonephritis (pauci-immune type), and the myeloperoxidase-specific ANCA (MPO-ANCA) titer was elevated at 1015
AAU. She was cured using steroid pulse therapy, combined with an angiotensin-converting-enzyme inhibitor and angiotensin-II receptor
blocker. (Korean J Med 76:S154-S157, 2009)
Keywords :Systemic sclerosis, ANCA, Crescentic glomerulonephritis, Renal crisis, Pulse therapy
