Peripheral Nervous System Involvement

  • Michael P. CollinsEmail author
  • P. James B. Dyck
Part of the Rare Diseases of the Immune System book series (RDIS)


Vasculitis is an uncommon but treatable and thus important cause of neuropathy. Vasculitic neuropathy can occur as an isolated entity (nonsystemic vasculitic neuropathy) but more frequently evolves in the setting of a systemic vasculitis. Polyarteritis nodosa, anti-neutrophil antibody-associated vasculitides (especially microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis), rheumatoid vasculitis, and cryoglobulinemic vasculitis are the systemic disorders most commonly associated with neuropathy. Both systemic vasculitic and nonsystemic vasculitic neuropathy usually present with a subacutely progressive, asymmetric/multifocal, painful, distal-predominant, axonal, sensory-motor phenotype. Available evidence suggests that nonsystemic and most systemic vasculitic neuropathies are mediated primarily by T-cell mechanisms, but immune complexes probably contribute. Evaluation of patients with suspected vasculitic neuropathy usually includes EMG, laboratory studies, and nerve/muscle biopsy. In patients lacking definite vasculitis in nerve biopsy, clinically probable vasculitic neuropathy can be diagnosed by recourse to supportive clinicopathologic findings. Vasculitic neuropathies occurring in the ANCA-associated vasculitides are treated in accordance with the underlying systemic vasculitis. About 50% of neuropathy-predominant primary systemic vasculitides are not controlled by initial corticosteroid monotherapy. IVIg or rituximab can induce remission in refractory or relapsing patients. Long-term outcome is reasonably good.


Vasculitis Neuropathy Antineutrophil cytoplasmic antibodies Nerve biopsy Pathology 



ANCA-associated vasculitides


American College of Rheumatology


Allograft inflammatory factor


Antinuclear antibody


Anti-neutrophil cytoplasmic antibody




Chapel Hill Consensus Conference


Chronic inflammatory demyelinating polyneuropathy






Connective tissue disease


Cytotoxic T lymphocyte




Diagnostic and Classification Criteria in Vasculitis Study


Direct immunofluorescence


Eosinophilic granulomatosis with polyangiitis




Erythrocyte sedimentation rate


European League Against Rheumatism


French Vasculitis Study Group


Giant cell arteritis


Granulomatosis with polyangiitis


Hereditary neuropathy with liability to pressure palsies


Inducible costimulator




Intravenous immunoglobulins


Mycophenolate mofetil


Matrix metalloproteinase


Microscopic polyangiitis






Nerve conduction studies


Nonsystemic vasculitic neuropathy


Polyarteritis nodosa


Plasma exchange


Proteinase 3


Randomized controlled trial


Rheumatoid factor


Systemic vasculitic neuropathy


Vascular endothelial growth factor


Vasculitic neuropathy


Varicella zoster virus


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Medical College of WisconsinMilwaukeeUSA
  2. 2.Mayo ClinicRochesterUSA

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