ANCA-related Crescentic Glomerulonephritis in a Patient with Scleroderma without Marked Dermatological Change and Malignant Hypertension

Mai TOMIOKA; Fumihiko HINOSHITA; Naoko MIYAUCHI; Yurika AKIYAMA; Shigeki SAIMA; Michiaki HIROE

doi:10.2169/internalmedicine.43.496

Renal Diseases

ANCA-related Crescentic Glomerulonephritis in a Patient with Scleroderma without Marked Dermatological Change and Malignant Hypertension

Mai TOMIOKA, Fumihiko HINOSHITA, Naoko MIYAUCHI, Yurika AKIYAMA, Shigeki SAIMA, Michiaki HIROE

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Keywords: systemic sclerosis sine scleroderma, ANCA, crescentic glomerulonephritis, renal crisis, pulse therapy

JOURNAL OPEN ACCESS

2004 Volume 43 Issue 6 Pages 496-502

DOI https://doi.org/10.2169/internalmedicine.43.496

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Abstract

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.

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