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Diagnosis and classification of central nervous system vasculitis

Authors
  • Hajj-Ali, Rula A.
  • Calabrese, Leonard H.1, 2, 3, 1, 4
  • 1 Cleveland Clinic Lerner College of Medicine of Case Western Reserve University
  • 2 Center for Vasculitis Care and Research
  • 3 Cleveland Clinic Foundation
  • 4 R. J. Fasenmyer Chair of Clinical Immunology
Type
Published Article
Journal
Journal of Autoimmunity
Publisher
Elsevier BV
Publication Date
Jan 01, 2014
Accepted Date
Nov 13, 2013
Volume
48-49
Pages
149–152
Identifiers
DOI: 10.1016/j.jaut.2014.01.007
Source
Elsevier
Keywords
License
Unknown

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.

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