Clinicopathological features of antineutrophil cytoplasmic antibodies-associated vasculitis in Japanese patients with IgA nephropathy |
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Authors: | M Shimizu T Wada N Sakai Y Izumiya K Furuichi T Misaki K Kobayashi S Goshima S Takeda H Yokoyama |
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Institution: | (1) First Department of Internal Medicine, School of Medicine, Kanazawa University, 13-1 Takara-machi, Kanazawa, Ishikawa 920-8641, Japan Tel. +81-762-65-2000 ext 3462; Fax +81-762-34-4250 e-mail: dialysis@medf.m.kanazawa-u.ac.jp, JP;(2) Division of Blood Purification, Kanazawa University Hospital, Kanazawa, Japan, JP;(3) Department of Internal Medicine, Inami General Hospital, Inami, Japan, JP;(4) Department of Internal Medicine, Kurobe City Hospital, Kurobe, Japan, JP |
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Abstract: | Antineutrophil cytoplasmic antibodies (ANCA) have been reported to be associated with systemic vasculitis. However, the roles
of ANCA subtypes in patients with IgA nephropathy remain to be fully investigated. We describe three Japanese patients with
IgA nephropathy complicated by ANCA-associated vasculitis. Two patients with IgG class ANCA developed rapidly progressive
renal failure and demonstrated mesangial proliferation with extensive extracapillary proliferation and segmental glomerular
necrosis. One patient with IgM class ANCA showed severe extrarenal symptoms, such as lung fibrosis and neuritis, in addition
to glomerular crescent formation. All three patients received immunosuppressive therapies, including corticosteroids and cyclophosphamide.
The two patients who received these treatments early showed improvement in urinary protein excretion and renal function, in
accordance with a decrease in the serum titer of ANCA. However, one patient in whom serum creatinine was already elevated
showed a poor response to the treatment. These results suggest that ANCA subtypes may participate in the pathogenesis of crescent
formation in patients with IgA nephropathy, and that early treatment with a combination of methylprednisolone pulse therapy,
oral prednisolone, and cyclophosphamide pulse therapy may be beneficial in these patients.
Received: November 2, 1999 / Accepted: February 8, 2000 |
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Keywords: | Antineutrophil cytoplasmic antibodies Crescentic glomerulonephritis Immunoglobulin A nephropathy Immunosuppressive therapy |
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