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Since both conditions share neurological manifestations, the following review will discuss the neurological aspects of both. This review aims to provide a comprehensive comparison of the pathogenesis, clinical manifestation and assessment, diagnostic workup, and treatment protocol for both PACNS and AAV with central nervous system involvement. To provide a comprehensive comparison and update, a literature review was conducted using PubMed and Ovid databases (Embase and Medline). Then, the references were retrieved, screened, and selected according to the inclusion and exclusion criteria. PACNS and AAV share similarities in clinical presentation and neurological symptoms, especially in terms of headache, focal deficits, and cognitive impairment. Additionally, both conditions may exhibit similarities in laboratory and radiological findings, making brain biopsy the gold standard for differentiation between the two conditions. Moreover, the treatment protocols for PACNS and AAV are nearly identical. Comparing PACNS and AAV with CNS involvement highlights the similarities in clinical presentation, radiological findings, and treatment protocols between the two conditions. Further research should focus on establishing a practical diagnostic protocol.<\/jats:p>","DOI":"10.1007\/s00296-023-05461-9","type":"journal-article","created":{"date-parts":[[2023,9,30]],"date-time":"2023-09-30T18:02:17Z","timestamp":1696096937000},"page":"211-222","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Primary angiitis of the CNS and ANCA-associated vasculitis: from pathology to treatment"],"prefix":"10.1007","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0629-8034","authenticated-orcid":false,"given":"Alaa","family":"Sherri","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1765-8170","authenticated-orcid":false,"given":"Mohamad Mahdi","family":"Mortada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2036-375X","authenticated-orcid":false,"given":"Joanna","family":"Makowska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8389-7399","authenticated-orcid":false,"given":"Anna","family":"Lewandowska-Polak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,9,30]]},"reference":[{"key":"5461_CR1","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1007\/s11910-022-01172-z","volume":"22","author":"G Mansueto","year":"2022","unstructured":"Mansueto G, Lanza G, Fisicaro F et al (2022) Central and peripheral nervous system complications of vasculitis syndromes from pathology to bedside: part 1\u2014central nervous system. 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