Diagnosis and classification of central nervous system vasculitis

J Autoimmun. Feb-Mar 2014;48-49:149-52. doi: 10.1016/j.jaut.2014.01.007. Epub 2014 Feb 1.

Abstract

Central nervous system vasculitis is one of the foremost diagnostic challenges in rheumatology. It results in inflammation and destruction of the vasculature within the CNS. When vasculitis is confined to brain, meninges or spinal cord, it is referred to as primary angiitis of the CNS. Secondary CNS vasculitis occurs in the setting of a systemic vasculitis, auto-inflammatory or infectious disease. Prompt and accurate diagnosis of CNS vasculitis is essential to prevent irreversible brain damage, and to secure precise treatment decisions. Progressive debilitating and unexplained neurological deficits, associated with abnormal cerebrospinal fluid is the typical picture of the disease. Biopsy of the brain remains the gold standard diagnostic test. The differential diagnosis of CNS vasculitis is highly diverse with a broad array of mimics at the clinical, radiographic and angiographic levels.

Keywords: CNS vasculitis; Primary angiitis of the central nervous system.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Brain* / immunology
  • Brain* / metabolism
  • Brain* / pathology
  • Diagnosis, Differential
  • Headache / diagnosis
  • Headache / immunology
  • Headache / pathology
  • Humans
  • Incidence
  • Inflammation / immunology
  • Inflammation / metabolism
  • Inflammation / pathology
  • Meninges* / immunology
  • Meninges* / metabolism
  • Meninges* / pathology
  • Retrospective Studies
  • Seizures / diagnosis
  • Seizures / immunology
  • Seizures / pathology
  • Sex Factors
  • Spinal Cord* / immunology
  • Spinal Cord* / metabolism
  • Spinal Cord* / pathology
  • Subarachnoid Hemorrhage / diagnosis
  • Subarachnoid Hemorrhage / immunology
  • Subarachnoid Hemorrhage / pathology
  • Vasculitis, Central Nervous System / classification*
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / immunology
  • Vasoconstriction / immunology