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Practice Guideline
. 2025 Oct;20(8):949-967.
doi: 10.1177/17474930251365861. Epub 2025 Jul 28.

Diagnosis and management of cerebral amyloid angiopathy: a scientific statement from the International CAA Association and the World Stroke Organization

Charlotte Cordonnier  1 , Catharina Klijn  2 , Eric Edward Smith  3 , Rustam Al-Shahi Salman  4 , Bart K Chwalisz  5 , Ellis van Etten  6 , Ryan T Muir  7 , Fabrizio Piazza  8 , Stefanie Schreiber  9   10 , Floris Hbm Schreuder  2 , Magdy Selim  11 , Ashkan Shoamanesh  12 , Anand Viswanathan  5 , Marieke Wermer  13 , Michael Zandi  14 , Andreas Charidimou  15 , Steven M Greenberg  5 , David Werring  16
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Free article
Practice Guideline

Diagnosis and management of cerebral amyloid angiopathy: a scientific statement from the International CAA Association and the World Stroke Organization

Charlotte Cordonnier et al. Int J Stroke. 2025 Oct.
Free article

Abstract

Cerebral amyloid angiopathy (CAA) is a well-recognized and challenging disease for neurologists and other clinicians caring for the rapidly aging worldwide population. CAA is a major cause of spontaneous lobar intracerebral hemorrhage (ICH), and can also cause transient focal neurological episodes, and convexity subarachnoid hemorrhage, CAA-associated ICH has a high mortality, morbidity, and recurrence rate. CAA can affect a wide range of clinical decisions including use of antithrombotic medications, safety for anti-β-amyloid peptide (Aβ) immunotherapy, and need for anti-inflammatory or immunosuppressive treatment. We present guidelines, intended to inform the approach to individuals with suspected CAA, written on behalf of the International CAA Association and the World Stroke Organization (WSO). We cover five areas selected for their relevance to practice: (1) diagnosis, testing, and prediction of intracerebral hemorrhage risk; (2) antithrombotic agents and vascular interventions; (3) vascular risk factors and concomitant medications; (4) treatment of CAA manifestations; and (5) diagnosis and treatment of CAA-related inflammation and vasculitis. The statement has been reviewed and approved by the Executive Committee of the WSO, and the International CAA Association.

Keywords: MRI; antithrombotic; brain bleed; brain microbleeds; cerebral hemorrhage; leukoaraiosis.

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Conflict of interest statement

Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr Cordonnier: None declared.Dr Klijn: steering committee of the cAPPricorn trial under a consulting services agreement between Alnylam Pharmaceuticals and Radboud university medical center.Dr Smith: research funding from Alnylam Pharmaceuticals, paid to the University of Calgary, to serve as a site for a clinical trial for cerebral amyloid angiopathy.Dr Al-Shahi Salman: None declaredDr Chwalisz: None declaredDr van Etten: local investigator for the cAPPricorn trial in CAA and member of the steering committee of the cAPPricorn trial for Alnylam Pharmaceuticals Inc.Dr Muir: None declared.Dr Piazza: Consulting services and educational activities, paid to the University of Milano Bicocca, with Alnylam Pharmaceuticals, Roche, Biogen, Alector, Lilly, Araclon. Inventor, without ownership, of the amyloid Ab antibody assay.Dr Schreiber: None declaredDr Schreuder: Local investigator for the cAPPricorn trial in CAA. Grant from Dutch Research Council (NWO ZonMw VENI grant; 09150162310154).Dr Selim: grants from the National Institutes of Health (NIH)/National Institute of Neurological Disorders and Stroke and National Institute of Aging; consulting services with Alnylam Pharmaceuticals, AegisCN, LLC and MedRhythms, Inc; stock holdings in NeuGel, Inc; and royalties Up-To-Date.Dr Shoamanesh: Steering Committees of the ASPIRE, ASPIRING and SATURN trials, Data Safety Monitoring Committee of the cAPPricorn trial (Alnylam Pharmaceuticals Inc.), (Co-)PI of the ENRICH-AF, SATURN-MRI and CoVasc-ICH trials, Steering Committee Co-Chair of the ANNEXa-I trial (AstraZeneca), consulting honoraria from Astrazeneca, Bayer AG, Bioxodes, Daiichi Sankyo, and VarmX.Dr Viswanathan: consultant Alnylam, Biogen.Dr Wermer: member of TRACK-D-CAA consortium funded by Alnylam Pharmaceuticals with payment to Leiden University Medical Center.Dr Zandi: honoraria for one lecture each for each of the four mentioned in the last 3 years: Norwegian Neurological Society; Copenhagen Neuropsychological Society, Rigshospitalet; Cygnet Healthcare, and GSK.Dr Charidimou: Consulting services with Alnylam Pharmaceuticals. Consulting services with Imperative Care.Dr Greenberg: steering committee of the cAPPricorn trial under a consulting services agreement between Alnylam Pharmaceuticals and Massachusetts General Hospital; royalties Up-To-Date.Dr Werring: local investigator for the cAPPricorn trial in CAA; has received honoraria for consulting and speaking from Alnylam. Professor Werring’s work is supported by the National Institute for Health and Care Research University College London Hospitals Biomedical Research Center.

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