Clinical and Pathological Findings in Microscopic Polyangiitis

概要

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Anti-neutrophil cytoplasmic autoantibody (ANCA) is useful in diagnosing vasculitis, while mechanisms by which ANCA causes vasculitis are not fully known. We studied 21 patients with microscopic polyangiitis (MPA) showing neuropathy with pathologically proven vasculitis in epoxy resin sections of biopsied nerve specimens. They consisted of myeloperoxidase (MPO) -ANCA-positive patients (n=15) and -negative patients (n=six). We compared clinical features and immunohistochemical findings of paraffin sections of the nerves between the two groups. Clinical features of neuropathy did not differ between the two groups. In MPO-ANCA-positive patients the titers fell in all patients undergoing corticosteroid therapy, but did not correlate with peak severity of neuropathic deficits. Immnohistochemical findings showed that neutrophils were conspicuous only in inflamed vessel walls in MPO-ANCA-positive group, and that little or no staining for immunoglobulin or complement could be demonstrated even in MPO-ANCA-positive group. These findings suggested that MPO-ANCA might initiate vasculitic neuropathy without immune deposition, so-called pauci-immune vasculitis, which is seen in kidney specimens from patients with MPA. Proinflammatory cytokines released from neutrophils may be important for pauci-immune vasculitis. Early treatment with corticosteroid is necessary for functional improvement in MPA patient.
兵庫医科大学の論文
2006-12-25