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Advancing Knowledge in CNS Vasculitis
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DOI：10.1212/NXI.0000000000200272.png)
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Only a few well-described cohorts of patients with primary CNS vasculitis (PCNSV; sometimes called primary angiitis of the CNS [PACNS]) have been published with enough follow-up to be able to really assess global and neurologic outcomes.(1,2) There is still no definitive clinical tool or biomarker to confirm the diagnosis of PCNSV. The commonly used 1988 diagnostic criteria from Calabrese and Malek, slightly modified in 2009 by Birnbaum and Hellmann, have not been thoroughly validated, but made sense and matched clinical practice and diagnostic reasoning.(3) A brain and/or leptomeningeal biopsy is the only way to definitively confirm CNS vasculitis, but a plethora of more frequent mimickers and causes for secondary CNS vasculitis have still to be ruled out, especially in patients with a diagnosis based on angiographic abnormalities and/or without obvious inflammatory CNS features on CSF analysis, gadolinium-enhancing lesions, or vessel wall enhancement on MRI.(3) A follow-up helps support the diagnosis, but possibly in the future, a minority of patients in these cohorts will be found to have a new genetic or infectious-triggered CNS condition. The list of PCNSV mimickers and secondary CNS vasculitis has been increasing steadily, with the identification of new genetically determined vasculopathies, such as deficiency in adenosine deaminase 2, and more sensitive tools, such as metagenomic next-generation sequencing, to detect rare CNS infections.(4) Hence, in real-world practice, as in the main published cohorts of PCNSV, only 30%-50% of reported patients had a brain biopsy, and only 50%-65% of the latter had biopsy-proven CNS vasculitis.(1,2) Additional information on the main subsets of PCNSV that have been described is also needed to better determine their respective outcomes and, in clinical practice, how best to treat them.
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PRIMARY ANGIITIS
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