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Primary angiitis of the central nervous system

What's new

Added 2023 ESO, 2021 AHA/ASA, and 2021 ACR guidelines for the diagnosis and management of primary angiitis of the central nervous system.

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of primary angiitis of the central nervous system are prepared by our editorial team based on guidelines from the European Stroke Organisation (ESO 2023), the American College of Radiology (ACR 2021), and the American Heart Association (AHA/ASA 2021). ...
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Diagnostic investigations

Diagnostic imaging: as per ESO 2023 guidelines, do not obtain MRA instead of digital subtraction angiography routinely in adult patients with suspected PACNS. Insufficient evidence regarding CTA.
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Diagnostic procedures

CSF analysis: as per ESO 2023 guidelines, insufficient evidence to recommend CSF analysis for pleocytosis and/or hyperproteinorrachia in patients with suspected PACNS.
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More topics in this section

  • Biopsy

  • Autopsy

Medical management

Immunosuppressive therapy: as per ESO 2023 guidelines, consider initiating immunosuppressants in addition to corticosteroids in most patients with PACNS, given the potential severity of PACNS, the relapsing course of the disease, and the well-known corticosteroid-related toxicity side effects in long-term administration. Consider administering corticosteroids alone in patients with milder disease phenotypes, decided in a multidisciplinary team with relevant expertise and/or an expert in the diagnosis and management of PACNS.
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