Non-systemic vasculitic neuropathy

Purpose of review This article reviews the literature on non-systemic vasculitic neuropathy, with emphasis on recent advances, summarizing the clinical presentation, diagnosis, pathology, treatment, and outcome of this condition, and speculating on its nosological status vis-a-vis the systemic vasculitides. Recent findings A new cohort of non-systemic vasculitic neuropathy patients was recently reported. Analysis of the clinical characteristics of this cohort demonstrated a higher incidence of painful, asymmetric, overlapping deficits than in previous studies. Extended follow-up revealed a high relapse rate, low risk of systemic spread, high incidence of chronic pain, relatively good neurological outcome, and low mortality rate. Analysis of therapeutic responses showed better outcomes with combination therapy than corticosteroid monotherapy. Another recent report proposed a role for magnetic resonance angiography in the diagnosis and follow-up of non-systemic vasculitic neuropathy. Recent pathological studies implicated proinflammatory cytokines and matrix metalloproteinase-9 in the mediation of vascular and axonal damage in non-systemic vasculitic neuropathy. Summary Non-systemic vasculitic neuropathy is one of many localized vasculitides, with involvement restricted to nerves and (possibly) muscles. Inclusion and exclusion criteria differ between reported cohorts. All require a nerve biopsy diagnostic of or suspicious for vasculitis and no extra-neuromuscular involvement. Patients typically present subacutely with a painful, multifocal/asymmetric, distal-predominant neuropathy. In the absence of clinical or laboratory evidence of systemic vasculitis or a condition predisposing to such, prognosis with treatment is good. Pathological data are supportive of a primary T-cell-mediated immunopathogenesis. Some patients classified as having non-systemic vasculitic neuropathy have a systemic vasculitis presenting with neuropathy;, in others, the disease is organ-specific.