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

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

    Tomioka M, Hinoshita F, Miyauchi N, Akiyama Y, Saima S, Hiroe M.

    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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