{"title":"Intracranial giant cell arteritis: a comprehensive systematic review.","authors":"Sagar Patel, Iva Okaj, Jessica Scott, Sukhreet Atwal, Colin Stark, Rabia Tahir, Nader Khalidi, Mats Junek","doi":"10.1093/rheumatology/keaf162","DOIUrl":null,"url":null,"abstract":"<p><strong>Objectives: </strong>Giant cell arteritis (GCA) is increasingly recognized to occur in intracranial vessels with unknown clinical ramifications. We identified all reported cases of intracranial GCA (ICGCA) in the literature to describe common presentations, investigations, treatments, and outcomes.</p><p><strong>Methods: </strong>We conducted a systematic review using MEDLINE, Embase and Pubmed databases to identify studies that reported cases of ICGCA. The study was registered on a systematic review database (PROSPERO 42023412373). We defined intracranial involvement as any vessel cranial to the dura mater that was confirmed by either histopathology or imaging. Data was summarized using descriptive statistics.</p><p><strong>Results: </strong>Of 1554 studies identified, 102 studies underwent full-text review. These studies included 340 patients with ICGCA. The median age was 73.7 (interquartile range (IQR) 71.9-77.3) and 46.9% patients were female. Presentations of ICGCA included stroke in 240 (70.6%) patients and isolated intracranial imaging or histologic changes in 67 (19.7%) patients. The most common vessels involved were 180 (52.9%) vertebrobasilar, 166 (48.8%) internal carotid and 49 (14.4%) ophthalmic arteries. Treatment was reported in 214 individuals. Glucocorticoids were administered to 210 (98.1%); tocilizumab, cyclophosphamide and methotrexate were the most common adjunctive medications. Of the 181 patients with reported follow-up outcomes, relapse occurred in 40 (22.1%) patients and 59 (32.6%) individuals died.</p><p><strong>Conclusion: </strong>Our findings suggest that ICGCA is not a rare entity and may represent a more severe manifestation of GCA. Optimal therapy for ICGCA is unknown. Structured prospective evaluation is needed to better understand this manifestation of GCA.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":""},"PeriodicalIF":4.7000,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Rheumatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/rheumatology/keaf162","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Objectives: Giant cell arteritis (GCA) is increasingly recognized to occur in intracranial vessels with unknown clinical ramifications. We identified all reported cases of intracranial GCA (ICGCA) in the literature to describe common presentations, investigations, treatments, and outcomes.
Methods: We conducted a systematic review using MEDLINE, Embase and Pubmed databases to identify studies that reported cases of ICGCA. The study was registered on a systematic review database (PROSPERO 42023412373). We defined intracranial involvement as any vessel cranial to the dura mater that was confirmed by either histopathology or imaging. Data was summarized using descriptive statistics.
Results: Of 1554 studies identified, 102 studies underwent full-text review. These studies included 340 patients with ICGCA. The median age was 73.7 (interquartile range (IQR) 71.9-77.3) and 46.9% patients were female. Presentations of ICGCA included stroke in 240 (70.6%) patients and isolated intracranial imaging or histologic changes in 67 (19.7%) patients. The most common vessels involved were 180 (52.9%) vertebrobasilar, 166 (48.8%) internal carotid and 49 (14.4%) ophthalmic arteries. Treatment was reported in 214 individuals. Glucocorticoids were administered to 210 (98.1%); tocilizumab, cyclophosphamide and methotrexate were the most common adjunctive medications. Of the 181 patients with reported follow-up outcomes, relapse occurred in 40 (22.1%) patients and 59 (32.6%) individuals died.
Conclusion: Our findings suggest that ICGCA is not a rare entity and may represent a more severe manifestation of GCA. Optimal therapy for ICGCA is unknown. Structured prospective evaluation is needed to better understand this manifestation of GCA.
期刊介绍:
Rheumatology strives to support research and discovery by publishing the highest quality original scientific papers with a focus on basic, clinical and translational research. The journal’s subject areas cover a wide range of paediatric and adult rheumatological conditions from an international perspective. It is an official journal of the British Society for Rheumatology, published by Oxford University Press.
Rheumatology publishes original articles, reviews, editorials, guidelines, concise reports, meta-analyses, original case reports, clinical vignettes, letters and matters arising from published material. The journal takes pride in serving the global rheumatology community, with a focus on high societal impact in the form of podcasts, videos and extended social media presence, and utilizing metrics such as Altmetric. Keep up to date by following the journal on Twitter @RheumJnl.