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Semin Neurol
DOI: 10.1055/s-0044-1791499
Review Article

Top 10 Clinical Pearls in Vasculitic Neuropathies

Peter Pacut
1   Department of Neurology, Virginia Commonwealth University, Richmond, Virginia
,
Kelly G. Gwathmey
1   Department of Neurology, Virginia Commonwealth University, Richmond, Virginia
› Author Affiliations
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Abstract

Vasculitic neuropathies are a diverse group of inflammatory polyneuropathies that result from systemic vasculitis (e.g., polyarteritis nodosa, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis), vasculitis resulting from rheumatological disorders (e.g., rheumatoid arthritis and Sjögren's syndrome), paraneoplastic conditions, viruses, and medications. Occasionally, vasculitis is restricted to the peripheral nerves and termed nonsystemic vasculitic neuropathy. Presenting with an acute or subacute onset of painful sensory and motor deficits, ischemia to individual peripheral nerves results in the classic “mononeuritis multiplex” pattern. Over time, overlapping mononeuropathies will result in a symmetrical or asymmetrical sensorimotor axonal polyneuropathy. The diagnosis of vasculitic neuropathies relies on extensive laboratory testing, electrodiagnostic testing, and nerve and/or other tissue biopsy. Treatment consists primarily of immunosuppressant medications such as corticosteroids, cyclophosphamide, rituximab, methotrexate, or azathioprine, in addition to neuropathic pain treatments. Frequently, other specialists such as rheumatologists, pulmonologists, and nephrologists will comanage these complex patients with systemic vasculitis. Prompt recognition of these conditions is imperative, as delays in treatment may result in permanent deficits and even death.

Keywords

vasculitic neuropathy - nonsystemic vasculitic neuropathy - mononeuritis multiplex


Publication History

Article published online:
30 September 2024

© 2024. Thieme. All rights reserved.

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