Corticosteroids lead to short-term improvement in cerebral amyloid angiopathy-related inflammation

Background: Cerebral amyloid angiopathy - related inflammation (CAA-ri) is an uncommon manifestation of CAA. Methods: Single-center, retrospective review of all charts with ICD-code 168.0 (CAA) from 2/2/2016-1/1/2020. Results: Of 152 CAA cases, 13 (8.6%) were consistent with CAA-ri. Corticosteroid-treatment led to short-term reduction in modified Rankin Scale scores (2.6 +/- 1.4 vs. 1.6 +/- 1.5; p = 0.01) and T2/FLAIR lesion volume (78.1 +/- 52.2 cm(3) vs. 30 +/- 30.9 cm(3), p < 0.01) as well as short-term improvement in post-treatment Clinical Global Impression - Global Change scores compared to pre-treatment scores (clinical: 6 +/- 1 vs. 2.6 +/- 1.3, p = 0.03; radiological: 4.6 +/- 1.9 vs. 1.2 +/- 0.4, p = 0.03). Interpretation: Corticosteroid-treatment leads to clinical and radiological short-term improvement (class IV evidence).