ANCA-related crescentic glomerulonephritis in a patient with scleroderma without marked dermatological change and malignant hypertension.
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.
Tomioka
Mai
M
Department of Medicine, International Medical Center of Japan, Tokyo.
Hinoshita
Fumihiko
F
Miyauchi
Naoko
N
Akiyama
Yurika
Y
Saima
Shigeki
S
Hiroe
Michiaki
M
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D005260:Female; D005921:Glomerulonephritis / Q000276:immunology*; D006801:Humans; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D051437:Renal Insufficiency / Q000188:drug therapy / Q000276:immunology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000276:immunology; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D011239:Prednisolone