Diagnosis and Treatment of Inflammatory Cerebral Amyloid Angiopathy

Purpose of review In this article, we discuss the current consensus approach to the evaluation and treatment of inflammatory cerebral amyloid angiopathy (CAA). We also outline critical knowledge gaps and identify questions for future research. Recent findings Recent cohort studies found that immunosuppressive treatment for inflammatory CAA was associated with higher probability of clinical and radiographic improvement and reduced risk of disease recurrence. A recent case series showed correlations between changes in cerebrospinal fluid levels of anti-Aβ autoantibodies, changes in microglial activation, and clinical and radiographic response to immunosuppressive treatment; these data provide supporting evidence for a pathophysiological link between inflammatory CAA and adverse events associated with Alzheimer’s disease (AD) immunotherapy treatments. Summary Despite many knowledge gaps, widely accepted methods for diagnosis and management of inflammatory CAA have emerged, including validated clinical and radiographic criteria and treatment with immunosuppressive medications. Still, there is considerable space for diagnosis and treatment paradigms to advance in tandem with our understanding of the disease. Efforts to optimize protocols for clinical and radiographic assessment, identify biomarkers at multiple stages of disease, and leverage insights from Alzheimer’s disease biomarkers and therapeutics will be pivotal to achieving these goals.