Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
Myeloperoxidase-Specific Anti-Neutrophil Cytoplasmic Autoantibodies - Related Scleroderma Renal Crisis Treated with Double-Filtration Plasmapheresis
Akiko OMOTE, Masahito MURAMATSU, Yoshiyuki SUGIMOTO, Shimako HOSONO, Ryuichiro MURAKAMI, Hitoshi TANAKA, Yoshiaki WATANABE, Hiroshi SANO, Katsumoto KATO
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Keywords: systemic sclerosis (SSc), necrotizing crescentic glomerulonephritis, interstitial pneumonia, mizoribine (MZR)
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1997 Volume 36 Issue 7 Pages 508-513

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Abstract

A 46-year-old female patient who was diagnosed with systemic sclerosis (SSc) developed rapidly progressive renal failure without elevation of blood pressure or plasma renin concentration. Renal biopsy revealed necrotizing crescentic glomerulonephritis (pauci-immune type) and the myeloperoxidase-speciflc anti-neutrophil cytoplasmic autoantibodies (MPO-ANCA) liter was found to be elevated to 669 EU/ml. Methylprednisolone (MP) pulse therapy followed by prednisolone (PSL) and mizoribine (MZR) did not suppress the progression of renal failure. Therefore, we started double-filtration plasmapheresis (DFPP) which effectively removed MPO-ANCA and prevented renal failure despite the relatively low dose of immunosuppressive agents.
(Internal Medicine 36: 508-513, 1997)

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