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Giant Cell Arteritis (Temporal Arteritis)

Practice Essentials

Giant cell arteritis (GCA), or temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that occurs in older persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications.
GCA is the most common form of systemic vasculitis in adults. Other names for GCA include arteritis cranialis, Horton disease, granulomatous arteritis, and arteritis of the aged.

GCA is classified as a large-vessel vasculitis
 but typically also involves medium and small arteries, particularly the superficial temporal arteries—hence the term temporal arteritis. In addition, GCA most commonly affects the ophthalmic, occipital, vertebral, posterior ciliary, and proximal vertebral arteries. Medium- and large-sized vessels that may be involved include the aorta and the carotid, subclavian, and iliac arteries.

Histopathologically, GCA is marked by transmural inflammation of the intima, media, and adventitia of affected arteries, as well as patchy infiltration by lymphocytes, macrophages, and multinucleated giant cells. Mural hyperplasia can narrow the arterial lumen, resulting in distal ischemia. (See Pathophysiology.)

Age and female sex are established risk factors for GCA, a genetic component seems likely, and infection may have a role (see Etiology). One school of thought considers GCA and polymyalgia rheumatica to be different manifestations of the same disease process, while others see them as closely related but different diseases.

Common signs and symptoms of GCA reflect the involvement of the temporal artery and other medium-sized arteries of the head and the neck and include visual disturbances, headache, jaw claudication, neck pain, and scalp tenderness. Constitutional manifestations, such as fatigue, malaise, and fever, may also be present. (See Presentation.)

GCA should always be considered in the differential diagnosis of a new-onset headache in patients 50 years of age or older with an elevated erythrocyte sedimentation rate. Temporal artery biopsy remains the criterion standard for diagnosis of this granulomatous vasculitis (see the image below). However, increasing evidence supports the use of imaging studies for diagnosis in patients at high clinical risk.

Hematoxylin- and eosin-stained superficial tempora

Hematoxylin- and eosin-stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of GCA shown here include intimal thickening with luminal stenosis, mononuclear inflammatory cell infiltrate with media invasion and necrosis, and giant cell formation in the media.

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Visual loss is one of the most significant causes of morbidity in GCA. Permanent visual impairment may occur in as many as 20% of patients, and, in some cases, GCA can cause bilateral blindness.
Newly recognized GCA should be considered a true neuro-ophthalmic emergency.

Prompt initiation of treatment may prevent blindness and other potentially irreversible ischemic sequelae of GCA.
Corticosteroids are the mainstay of therapy. In steroid-resistant cases, drugs such as tocilizumab, cyclosporine, azathioprine, or methotrexate may be used as steroid-sparing agents. The typical patient with GCA remains on steroid therapy for roughly 2 years. (See Treatment and Medication.)

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