Non-systemic Vasculitic Neuropathy

Non-systemic Vasculitic Neuropathy

非全身性血管炎性ニューロパチー（non-systemic vasculitic neuropathy）は末梢神経に限局した血管炎であり，顕微鏡的多発血管炎や好酸球性多発血管炎性肉芽腫症と並んで血管炎によるニューロパチーの主要な原因である。病態に関する検討は十分なされておらず，全身性血管炎との連続性に関しても明らかになっていない。今後，本疾患の病態を解明することによって血管炎の中での位置づけを明らかにするとともに，診療に役立つバイオマーカーを開発する必要がある。

Abstract

Non-systemic vasculitic neuropathy (NSVN) is a vasculitis confined to the peripheral nervous system. It is one of the most common causes of vasculitic neuropathy, along with microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). It remains unclear whether this disease is truly an isolated nosological entity, or part of the spectrum of systemic vasculitides. Anti-neutrophil cytoplasmic antibodies (ANCA) are negative in NSVN even though the size of affected vessels in this disease is similar to that in ANCA-associated vasculitis. Although the concept of single organ vasculitis was added to the 2012 revised International Chapel Hill Consensus Conference (CHCC) nomenclature of vasculitides, a description of NSVN has not yet been incorporated. Further studies are needed to elucidate the pathogenesis of NSVN, particularly a search for causative antibodies or participation of complement pathways. These studies may help clarify the position of NSVN along the spectrum of vasculitides based on CHCC nomenclature.