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

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.