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Primary angiitis of the central nervous system

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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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Guidelines

1.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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2.Diagnostic procedures

Cerebrospinal fluid analysis: insufficient evidence to recommend CSF analysis for pleocytosis and/or hyperproteinorrachia in patients with suspected PACNS.
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  • Biopsy

  • Autopsy

3.Medical management

Immunosuppressive therapy: 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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  • Management of stroke (endovascular thrombectomy)