Vasculitic neuropathies

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By Comana M Cioroiu MD

The vasculitides are a group of heterogeneous disorders that present with a variable and complex clinical picture. Debates over clinical versus pathological approaches to classification abound in the literature, all these, with recognized limitations given the variable clinical presentations and the overlap between the recognized diagnostic entities. Peripheral neuropathy is an important, and often the presenting clinical feature of the vasculitides. Its recognition can be critical to attain an early diagnosis in these disorders where the ultimate outcome can be greatly influenced by early therapeutic intervention.

  • Neuropathy related to vasculitis can occur as either a primary condition (in the setting of systemic vasculitis) or secondary to a range of diseases including infection and connective tissue disorders. Localized nonsystemic vasculitic neuropathies include diabetic lumbosacral radiculoplexus neuropathy (DLRPN) and nondiabetic lumbosacral radiculoplexus neuropathy (LRPN).
  • Vasculitic neuropathies can be classified according to clinical features or histopathologically based on the size of involved vessels.
  • Diagnostic tests include electrodiagnostic studies demonstrating an asymmetric non-length dependent axonal neuropathy and nerve biopsy when necessary.
  • Management involves institution of immunosuppressive therapy and removal of the inciting agent when present.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References Cited