Giant Cell Arteritis (GCA) is a chronic systemic granulomatous vasculitis of large and medium sized arteries. While classical manifestations of GCA are due to involvement of the carotid artery and its branches, extra cranial large vessel involvement is increasingly recognised. The diagnosis of large vessel involvement in GCA may be challenging. These patients often have a paucity of symptoms, present atypically and their temporal artery biopsies are more frequently negative. Mortality is increased in the presence of aortic and thoracic aneurysms, however much debate exists on the best imaging modality to screen for their presence and on the optimal schedule and duration of screening.
Glucocorticoids remain the cornerstone of therapy in GCA. Treatment related adverse events occur in the majority and glucocorticoid resistance is not uncommon. Prospective randomised controlled trials on traditional disease modifying agents are few and in some cases have provided conflicting results. Newer biologic agents hold promise but further evidence is needed.
The case to be presented demonstrates the inherent difficulties in the diagnosis and subsequent management of patients with GCA and extra cranial large vessel vasculitis.
Disclosure of Interest None Declared