High risk and low prevalence diseases: Giant cell arteritis

Giant cell arteritis (GCA) is a serious condition that carries with it a high rate of morbidity.

This review highlights the pearls and pitfalls of GCA in adult patients, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence.

GCA is an immune-mediated vasculitis of medium-sized vessels that primarily affects those over the age of 50 years. Patients can present with a variety of signs and symptoms, including headache, vision changes, and systemic findings such as fever. Findings including jaw and limb claudication, vision changes, and temporal artery abnormalities are specific for diagnosis. While there are no highly sensitive features of the history and examination, the disease should be suspected in patients over the age of 50 years with vision changes, new headache, temporal artery abnormalities, or jaw claudication, especially in the setting of systemic symptoms. Inflammatory markers including erythrocyte sedimentation rate and c-reactive protein in combination are sensitive but not specific for GCA. Delay in diagnosis is associated with vision loss and other complications including aortitis. If suspected, the emergency physician should administer steroids and consult the ophthalmology and rheumatology specialists.

An understanding of GCA can assist emergency clinicians in diagnosing and managing this potentially dangerous disease.