Primary angiitis of the central nervous system in adults
- Rula A Hajj-Ali, MD
Rula A Hajj-Ali, MD
- Associate Professor of Medicine
- Cleveland Clinic Lerner College of Medicine
- Leonard H Calabrese, DO
Leonard H Calabrese, DO
- Professor of Medicine
- Cleveland Clinic Lerner College of Medicine of Case Western Reserve University
- Section Editors
- Gene G Hunder, MD
Gene G Hunder, MD
- Section Editor — Vasculitis
- Emeritus Consultant
- Professor Emeritus
- Mayo Clinic College of Medicine
- Scott E Kasner, MD
Scott E Kasner, MD
- Section Editor — Stroke
- Professor of Neurology
- University of Pennsylvania School of Medicine
Central nervous system (CNS) vasculitis refers to a broad spectrum of diseases that result in inflammation and destruction of the blood vessels of the brain, spinal cord, and meninges. Angiitis, a synonym for vasculitis, refers generally to blood vessels on both the arterial and venous sides of the circulation.
Primary angiitis of the CNS (PACNS) is the preferred name for vasculitis that is confined to the CNS. CNS vasculitis is considered secondary when it occurs in the context of a systemic inflammatory disease, such as a systemic vasculitis or systemic lupus erythematosus (SLE), or an infectious process such as varicella zoster virus.
PACNS predominantly affects small- and medium-sized arteries of the brain parenchyma, spinal cord, and leptomeninges, resulting in symptoms and signs of CNS dysfunction. It is defined by inflammation of the cerebral vasculature without angiitis in other organs.
The protean manifestations of PACNS, along with the nonspecificity of available investigational modalities, pose a challenge for accurate diagnosis. Clinicians caring for patients with suspected PACNS should be familiar with its mimics to avoid misdiagnosis. PACNS can be mimicked closely in both its clinical presentation and radiologic manifestations by a number of other disorders. The most frequent mimic of PACNS is a group of disorders known collectively as the reversible cerebral vasoconstriction syndromes (RCVS). (See "Reversible cerebral vasoconstriction syndromes".)
The clinical characteristics, diagnosis, and treatment of PACNS will be reviewed here. PACNS in children is discussed separately. (See "Vasculitis in children: Classification and incidence", section on 'Primary angiitis of the central nervous system'.)
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- CLINICAL MANIFESTATIONS
- DIAGNOSTIC APPROACH
- When to suspect the diagnosis
- Alternative diagnoses
- - Laboratory testing to exclude alternative diagnoses
- - Lumbar puncture
- - Neuroimaging
- MR angiography and CT angiography
- Conventional angiography
- - Brain and leptomeningeal biopsy
- Selecting patients for brain biopsy
- Establishing the diagnosis
- Initial therapy of suspected PACNS
- Treatment of PACNS
- - Glucocorticoids
- - Cyclophosphamide
- - Rituximab
- Prophylactic therapies
- - Osteoporosis prevention
- - Opportunistic infections
- - Other
- Monitoring treatment
- Duration of therapy and management of recurrent disease
- - Glucocorticoid taper
- - Discontinuing cyclophosphamide
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS