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
