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Review
. 2024 Jul;20(7):4999-5008.
doi: 10.1002/alz.13826. Epub 2024 Jun 17.

Cerebral amyloid angiopathy and the immune system

Danielle Munsterman  1 , Sarina Falcione  1 , Rebecca Long  1 , Roobina Boghozian  1 , Twinkle Joy  1 , Richard Camicioli  1 , Eric E Smith  2 , Glen C Jickling  1
Affiliations
Review

Cerebral amyloid angiopathy and the immune system

Danielle Munsterman et al. Alzheimers Dement. 2024 Jul.

Abstract

Cerebral amyloid angiopathy (CAA) is characterized by the accumulation of amyloid protein in the walls of cerebral blood vessels. This deposition of amyloid causes damage to the cerebral vasculature, resulting in blood-brain barrier disruption, cerebral hemorrhage, cognitive decline, and dementia. The role of the immune system in CAA is complex and not fully understood. While the immune system has a clear role in the rare inflammatory variants of CAA (CAA related inflammation and Abeta related angiitis), the more common variants of CAA also have immune system involvement. In a protective role, immune cells may facilitate the clearance of beta-amyloid from the cerebral vasculature. The immune system can also contribute to CAA pathology, promoting vascular injury, blood-brain barrier breakdown, inflammation, and progression of CAA. In this review, we summarize the role of the immune system in CAA, including the potential of immune based treatment strategies to slow vascular disease in CAA and associated cognitive impairment, white matter disease progression, and reduce the risk of cerebral hemorrhage. HIGHLIGHTS: The immune system has a role in cerebral amyloid angiopathy (CAA) which is summarized in this review. There is an inflammatory response to beta-amyloid that may contribute to brain injury and cognitive impairment. Immune cells may facilitate the clearance of beta-amyloid from the cerebral vasculature. Improved understanding of the immune system in CAA may afford novel treatment to improve outcomes in patients with CAA.

Keywords: Abeta related angiitis; CAA related inflammation; cerebral amyloid angiopathy; hemorrhagic stroke; immune response; microbleeds.

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Conflict of interest statement

Dr Eric E. Smith reports consulting for Eli Lilly and Alnylam and is a site investigator for a clinical trial sponsored by Biogen. All other authors declare no conflicts of interest. Author disclosures are available in the Supporting Information.

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