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1: Intern Med. 2004 Jun;43(6):496-502. Links

ANCA-related crescentic glomerulonephritis in a patient with scleroderma without marked dermatological change and malignant hypertension.

Department of Medicine, International Medical Center of Japan, Tokyo.

A 64-year-old woman with scleroderma without marked dermatological change developed anti-neutrophil cytoplasmic autoantibody (ANCA)-related renal failure. She had neither malignant hypertension nor elevation of plasma renin concentration. Renal biopsy showed crescentic glomerulonephritis (pauci-immune type) and the myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA) titer was found to be elevated to 757 IU/ml. Methylprednisolone pulse therapy followed by oral prednisolone effectively suppressed renal failure and lowered the MPO-ANCA titer. We believe this is a rare case of ANCA-related renal failure in a patient with scleroderma without marked dermatological change.

PMID: 15283187 [PubMed - indexed for MEDLINE]

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