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Adult primary central nervous system vasculitis: an update
Carlo Salvarani1, Robert D Brown, Gene G Hunder
1Unit of Rheumatology, Arcispedale S. Maria Nuova, Reggio Emilia, Italy. salvarani.carlo@asmn.re.it
Current Opinion in Rheumatology
|December 14, 2011
Summary
Recent advances in diagnosing and treating adult primary central nervous system vasculitis (PCNSV) improve patient outcomes. New criteria and neuroimaging aid diagnosis, revealing PCNSV
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Primary central nervous system vasculitis (PCNSV) is a rare condition affecting blood vessels in the brain and spinal cord.
- Accurate diagnosis and timely treatment are crucial for preventing neurological damage.
Purpose of the Study:
- To review recent advancements in the clinical assessment, diagnosis, and treatment of adult PCNSV.
- To highlight new diagnostic criteria and imaging techniques.
- To discuss the evolving understanding of PCNSV heterogeneity and prognosis.
Main Methods:
- Literature review of recent studies on PCNSV.
- Analysis of new diagnostic criteria and neuroimaging advancements.
- Examination of histopathological findings and clinical subsets.
Main Results:
- New diagnostic criteria proposed to reduce misdiagnosis, especially differentiating from reversible cerebral vasoconstriction syndromes.
- Advances in neuroimaging enhance the ability to distinguish inflammatory from non-inflammatory vascular lesions.
- PCNSV is recognized as a heterogeneous disorder with varied histopathology, including associations with amyloid angiopathy.
- Recent studies indicate a more favorable prognosis, with 81% of patients showing good response to therapy and favorable outcomes, contrasting with earlier reports.
Conclusions:
- Improved understanding of the PCNSV spectrum and its subsets aids in earlier recognition.
- Earlier diagnosis and treatment can prevent irreversible neurological damage and improve patient outcomes.
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