血管炎性末梢神経障害の病理

Pathology of Vasculitic Neuropathies

血管炎性ニューロパチーの確定診断には神経生検による組織診断が重要である。組織所見は血管壁内の細胞浸潤を伴う血管構造の破壊と急性の軸索変性が典型的であるが，採取した標本内に血管炎の直接の証拠が得られないこともあるので，虚血を示唆する間接的な所見にも注意する。そのほか浸潤細胞の種類や炎症血管のサイズ，肉芽腫の有無などが鑑別診断に役立つ。

Abstract

Prompt diagnosis of vasculitis is necessary because neuropathy may be the first symptom of vasculitic syndrome, and it is crucial for the management of patients. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, rheumatoid vasculitis, cryoglobulinemia, Sjogren's syndrome, and non-systemic vasculitic neuropathy are disorders frequently associated with vasculitic neuropathy. Pathological features such as the size of the involved vessels and the type of inflammatory cells, combined with general symptoms and laboratory data, may contribute to the specific diagnosis. ANCA may be involved in the pathogenesis of microscopic polyangiitis, and eosinophilic toxins may injure the nerve fibers in some patients with eosinophilic granulomatosis with polyangiitis. We detected B-cell follicle-like structures adjacent to the inflamed vessels in rheumatoid vasculitis, which may be associated with the pathogenesis.