Vasculitis of the nervous system

Curr Opin Neurol. 2004 Jun;17(3):317-36. doi: 10.1097/00019052-200406000-00014.


Purpose of review: Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. When unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability making this a disorder of paramount importance to all clinicians.

Recent findings: Remarkable progress has been made in the pathogenesis, diagnosis, and treatment of vasculitis of the central (CNS) and peripheral nervous system (PNS). The classification of vasculitis affecting the nervous system includes (1) Systemic vasculitis disorders (necrotizing arteritis of the polyarteritis type, hypersensitivity vasculitis, systemic granulomatous vasculitis, giant cell arteritis, diverse connective tissue disorders; viral, spirochete, fungal, and retroviral infection; (2) Paraneoplastic disorders; (3) Amphetamine abuse; (4) Granulomatous angiitis of the brain; (5) Isolated peripheral nerve vasculitis, each in the absence of systemic involvement; and (6) diabetes mellitus, associated wtih inflammatory PNS vasculopathy.

Summary: Vasculitis is diagnosed with assurance after intensive evaluation. Successful treatment follows ascertainment of the specific vasculitic disorder and the underlying cytochemical mechanism of pathogenesis. Clinicians must choose from among the available immunomodulating, immunosuppressive, and targeted immunotherapies, unfortunately without the benefit of prospective clinical trials, tempered by the recognition of all of the possible medication related side effects.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Azathioprine / therapeutic use
  • Connective Tissue Diseases / complications
  • Cyclophosphamide / therapeutic use
  • Diagnosis, Differential
  • Giant Cell Arteritis / diagnosis
  • Giant Cell Arteritis / pathology
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / pathology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Infections / complications
  • Peripheral Nervous System Diseases
  • Polyarteritis Nodosa / diagnosis
  • Polyarteritis Nodosa / pathology
  • Prednisone / therapeutic use
  • Vasculitis / diagnosis*
  • Vasculitis / pathology*
  • Vasculitis / therapy
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / pathology*
  • Vasculitis, Leukocytoclastic, Cutaneous / diagnosis
  • Vasculitis, Leukocytoclastic, Cutaneous / pathology


  • Immunosuppressive Agents
  • Cyclophosphamide
  • Azathioprine
  • Prednisone