Diagnosis and classification of central nervous system vasculitis.
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.
Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Center for Vasculitis Care and Research, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA. Electronic address: hajjalr@ccf.org.
Rula A
RA
Hajj-Ali
Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, R. J. Fasenmyer Chair of Clinical Immunology, 9500 Euclid Avenue, Cleveland, OH, USA. Electronic address: calabrl@ccf.org.
Leonard H
LH
Calabrese
D001921:Brain / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D003937:Diagnosis, Differential; D006261:Headache / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D006801:Humans; D015994:Incidence; D007249:Inflammation / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D008578:Meninges / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D012189:Retrospective Studies; D012640:Seizures / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D012737:Sex Factors; D013116:Spinal Cord / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D013345:Subarachnoid Hemorrhage / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D020293:Vasculitis, Central Nervous System / Q000145:classification* / Q000175:diagnosis* / Q000276:immunology; D014661:Vasoconstriction / Q000276:immunology
CNS vasculitis; Primary angiitis of the central nervous system