Intracranial Arteriopathy and Ischemic Brain Injury in Adults With Sickle Cell Disease

Cerebrovascular injury is a complication of sickle cell disease (SCD), but the spectrum of MRI/MRA abnormalities in adults remains incompletely characterized. We described imaging abnormalities in a consecutive adult SCD clinic cohort undergoing MRI/MRA and examined clinical risk factors and intracranial arteriopathy features associated with parenchymal abnormalities.

We retrospectively studied 527 consecutive adults with confirmed SCD who underwent brain MRI/MRA as part of routine follow-up at a tertiary SCD clinic, not restricted to patients with a history of stroke or TIA. Parenchymal outcomes included cortical, lacunar, watershed, and large-vessel territorial infarcts, Fazekas score >1, and global cortical atrophy. For each ischemic or atrophy-related outcome, Model A examined associations with predefined clinical and laboratory factors. Model B added steno-occlusive arteriopathy (SOA) and dilative intracranial arteriopathy (DIA) to evaluate whether vascular imaging features were associated with parenchymal abnormalities beyond clinical factors alone.

Mean age was 33.0 ± 12.6 years; 55.6% were female. Ischemic abnormalities included cortical infarcts (13.1%), lacunar infarcts (9.7%), watershed infarcts (5.3%), and large-vessel territorial infarcts (3.4%). SOA was present in 9.3% and was associated with Fazekas score >1, lacunar infarcts, and global cortical atrophy in multivariable models. In targeted analyses, SOA was also associated with watershed and large-vessel territorial infarcts.

In a consecutive adult SCD cohort undergoing routine MRI/MRA, ischemic and atrophy-related abnormalities were frequent and heterogeneous overall. SOA, but not DIA, was associated with several parenchymal injury patterns, supporting the potential role of intracranial vascular imaging in refining cerebrovascular phenotyping in adults with SCD.