Temporal arteritis

Yin Allison Liu MD PhD (

Dr. Liu of University of California, Davis has no relevant financial relationships to disclose.

)
Originally released May 14, 1996; last updated July 25, 2020; expires July 25, 2023

Overview

Temporal arteritis is the most common form of systemic vasculitis in the elderly. The etiology is unknown. Temporal arteritis can result in systemic, neurologic, and ophthalmological complications. When it presents as arteritic ischemic optic neuropathy, it becomes a true visual emergency, as the other eye may become involved within days if not treated promptly. In addition, if left untreated, these patients are at increased risk for ischemic stroke. High-dose corticosteroids are the mainstay of acute and chronic treatment. Other adjunctive immunosuppressants are also used as steroid-sparing agents. In this update, the author reviews advances in diagnosis and treatment for temporal arteritis.

Key points

 

• Temporal arteritis is a systemic vascular disease in which inflammation, usually with giant cells, affects the internal elastic lamina, adventitia, and adjacent media of medium and large arteries with a predilection for those in the head and neck.

 

• It is a disease almost exclusively in the elderly (older than 60 years) and usually presents with headache along with manifestations of ischemia in tissues of the head including most commonly pain, ulceration, and necrosis involving the scalp and oral mucosa.

 

• Ischemia of the temporalis and masseter muscles produces a distinctive syndrome called jaw claudication with crescendo pain in the mandibular region while chewing solid food and gradual relief after cessation of muscular activity in the involved muscles.

 

• The most common serious complication is vision loss from ischemic optic neuropathy or central retinal artery occlusion.

 

• Ischemic stroke can occur secondary to emboli from involved extracranial carotid and vertebral arteries.

 

• Serious complications can be prevented by treatment with systemic corticosteroids in adequate doses over prolonged periods.

Historical note and terminology

Credit for the earliest complete description of temporal arteritis must be accorded to Horton and colleagues from the Mayo Clinic. The following is their succinct clinical description derived from examining 2 patients:

 

Both patients were admitted to the Clinic in the spring of 1931 because of fever, weakness, anorexia, loss of weight, anemia, mild leukocytosis, and painful, tender areas over the scalp and along temporal vessels. These manifestations had been present for 4 weeks to 6 weeks. Temporal artery biopsy in these 2 cases yielded granulomatous arteritis and periarteritis (Horton et al 1932).

Since this report, temporal artery biopsy has remained as the mainstay of pathologic diagnosis.

Another painful syndrome, now called "polymyalgia rheumatica," shares with temporal arteritis the strong predilection for the elderly and the association with signs and symptoms of systemic illness (Barber 1957). In the mid-1950s, the concept that temporal arteritis and polymyalgia rheumatica are 2 clinical manifestations of the same basic disease process was introduced (Paulley and Hughes 1960). Hamrin and Ostberg both provided landmark publications, the former reporting his series of 93 cases of polymyalgia rheumatica with analysis of the relationship with temporal arteritis and underlying vascular pathology, and the latter reporting an autopsy study that revealed the link between the clinical syndrome called "polymyalgia rheumatica and giant cell arteritis of the aorta and its main branches" (Hamrin 1972; Ostberg 1973).

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