A Review of Intracranial GCA Save

A comprehensive review in Rheumatology on intracranial giant cell arteritis (icGCA) stresses that GCA is both an intracranial and extracranial large vessel vasculitis, with the former having unique presentations, and outcomes.

A systematic review included 102 studies and 340 patients with icGCA. The median age was 73.7 years with 47% patients being female.

While stroke is thought to be uncommon (3–7%) in GCA, in this study of icGCA patients, stroke was the most common presesentation (71%) with evidence of isolated intracranial imaging or histologic changes in nearly 20% of patients. 

The most common vessels involved were vertebrobasilar (53%), internal carotid (49%) and ophthalmic arteries (14%).  While nearly all received glucocorticoids (98%); steroid sparing agents were commonly used as well -  tocilizumab, cyclophosphamide and methotrexate

Ultimately, relapse occurred in 22% and 33% died.

Given these surprising outcomes, there is room for study to determine the optimal therapy for icGCA.  These findings support recent guidelines suggesting the need for extended vascular imaging when GCA is newly diagnosed or problematic.