title: [Non-systemic Vasculitic Neuropathy].
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.
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine.
authors.forename: Haruki
authors.initials: H
authors.lastname: Koike
mesh_terms: D001706:Biopsy; D006801:Humans; D009460:Neurologic Examination; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000209:etiology; D014657:Vasculitis / Q000150:complications*