Intracranial Involvement in Giant Cell Arteritis Linked to Stroke and Increased Mortality

A significant number of patients with giant cell arteritis (GCA) experience involvement of the intracranial vessels, a condition that appears to carry a heightened risk for stroke and poor outcomes, according to findings from a systematic review published in Rheumatology.

While GCA is classically considered a disease of the large extracranial vessels, emerging evidence indicates that its reach may extend into the brain’s vasculature. To better understand this manifestation, researchers conducted a comprehensive systematic review of published literature to characterize the clinical presentation, diagnosis, treatment, and outcomes of intracranial GCA (ICGCA).

The researchers searched MEDLINE, Embase, and PubMed for studies of all design types published until February 2023. They identified 102 studies that collectively involved 340 patients with confirmed ICGCA based on imaging or histopathologic evidence. Patients were aged a median of 73.7 years and nearly half (46.9%) were women.

Stroke was the predominant presenting feature of ICGCA, reported among 70.6% of patients. Among these individuals, motor deficits (44.6%), speech disturbances (33.8%), and cerebellar symptoms (33.8%) were common. A smaller subset of patients (n=67) showed only radiographic or histologic signs of disease without clinical symptoms, suggesting a possible underrecognized burden of silent intracranial inflammation in GCA.

Results from imaging studies revealed that the vertebrobasilar arteries were most frequently involved (52.9%), followed by the internal carotid (48.8%) and ophthalmic arteries (14.4%). The researchers frequently observed vessel abnormalities such as stenosis/occlusion (67.8%) and wall thickening (44.9%).

Treatment strategies varied widely, with nearly all patients (98.1%) receiving glucocorticoids and roughly one-third receiving additional immunosuppressive agents such as cyclophosphamide, methotrexate, or tocilizumab. Antiplatelet therapy was used among 34.6% of patients and 12.6% underwent revascularization procedures. However, no standardized treatment protocol for ICGCA was found among the included studies.

Despite aggressive therapy, outcomes were poor among patients with intracranial involvement. Out of the 181 patients with follow-up data, 32.6% died, primarily from stroke recurrence or disease progression. Additionally, 22.1% experienced disease relapse and many presented with complications such as new strokes or permanent vision loss.

Study limitations include publication bias, heterogeneity among diagnostic approaches, and difficulty differentiating vasculitis from atherosclerotic disease on imaging.

“These findings indicate that the presence of new neurologic symptoms in the context of an existing GCA diagnosis should be comprehensively evaluated for stroke and ICGCA,” the study authors concluded.

Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of disclosures.

This article originally appeared on Rheumatology Advisor