eMedicine Specialties > Radiology > Vascular/Interventional

Temporal Arteritis

Author: Anthony W Allen, MD, Chief, Interventional Radiology, Brooke Army Medical Center; Associate Professor of Radiology, Uniformed Services University of the Health Sciences
Coauthor(s): Timothy Biega, MD, Staff Physician, Department of Radiology, Tripler Regional Medical Center; Manish K Varma, MD, Chief of Interventional Radiology, Department of Radiology, Tripler Army Medical Center
Contributor Information and Disclosures

Updated: Oct 2, 2008

Introduction

Background

Temporal arteritis, or giant cell arteritis, is a common systemic vasculitis of unknown etiology. In 1890, Hutchinson originally described the condition as inflamed and swollen temporal arteries. In 1932, Horton expanded the definition. In general, temporal arteritis can be thought of as a vasculitis involving medium-to-large arteries originating from the aorta. Although it was originally believed to be a rare entity, it is more commonly recognized today.^1,2,3,4,5

Pathophysiology

The exact cause of temporal arteritis (giant cell arteritis) is not well delineated. It is characterized by a granulomatous inflammatory process that is most pronounced along the internal elastic lamina of arterial walls. Temporal artery biopsy demonstrates a predominance of mononuclear cells or multinucleated giant cells with fragmentation of the intima. Inflammation may be followed by intimal proliferation and eventual stenosis or occlusion of the involved arterial segment.

The clinical findings are a result of decreased blood flow and include jaw or tongue claudication; extremity stiffness; scalp tenderness; and visual changes with anterior ischemic optic neuropathy (AION), amaurosis, or optic atrophy leading to blindness in as many as 60% of patients.

Frequency

United States

Temporal arteritis (giant cell arteritis) occurs in an estimated 15-30 per 100,000 persons. It almost exclusively affects individuals older than 50 years and is believed to have a prevalence as high as 1 case per 500 individuals in this age group.

Mortality/Morbidity

To the author's knowledge, no controlled studies have been performed to compare patients with untreated temporal arteritis (giant cell arteritis) and patients with treated arteritis. However, definitive evidence suggests that mortality rates in treated patients and those in untreated patients do not differ.

Race

The incidence of temporal arteritis (giant cell arteritis) is lower in blacks than in whites.

Sex

The female-to-male ratio for temporal arteritis (giant cell arteritis) is 4-6:1.

Age

Although temporal arteritis (giant cell arteritis) occurs almost exclusively in patients older than 50 years, well-documented cases have been reported in patients as young as 40 years.

Anatomy

Temporal arteritis (giant cell arteritis) can affect any medium or large artery, but the clinical signs and symptoms are usually related to the inflammation that occurs in the branches of the external carotid artery.⁶ Temporal arteritis is caused by inflammation of the internal elastic lamina of the arterial wall. Intradural cranial arteries have no elastic lamina. For this reason, temporal arteritis seldom affects the cerebral circulation.

In the region of the parotid gland, the external carotid artery terminates by dividing into the superficial temporal and maxillary arteries. The superficial temporal artery crosses the zygomatic process of the temporal bone and divides into frontal and parietal branches that traverse the scalp. The superficial temporal artery typically does not develop atherosclerotic plaques, and the vessel is reportedly observed on 88% of cerebral angiograms.⁷ In addition, angiography can demonstrate areas of constriction, beading, and microaneurysm formation that are fairly specific for temporal arteritis. The most common sites for abnormalities to occur anatomically and on imaging studies are in the distal subclavian, proximal axillary, brachial, brachiocephalic, and femoral arteries.

Presentation

Multiple symptoms may be present in the patient with temporal arteritis and are usually attributable to decreased blood flow in the affected anatomic area. These symptoms include headache, which may be unilateral; scalp tenderness; jaw and/or tongue claudication; and changes in vision, including blindness. Constitutional symptoms may be present as well, and these are likely the result of the inflammatory nature of the disease. Fever, anorexia, and stiffness of the neck, trunk, and extremities may be present.

In 1990, the American College of Rheumatology established criteria for the classification of temporal arteritis. In one study, investigators compared 214 patients with temporal arteritis with 593 control subjects and determined that if patients had 3 of the 5 criteria, temporal arteritis could be diagnosed with a sensitivity of 93.5% and a specificity of 91.2%. The 5 criteria are as follows: (1) age older than 50 years, (2) new onset of localized headache, (3) temporal artery tenderness or decreased temporal arterial pulse, (4) increased erythrocyte sedimentation rate (>50 mm/h), and (5) arterial biopsy showing necrotizing arteritis characterized by a predominance of mononuclear cell infiltrates or a granulomatous process.^8,9

Preferred Examination

Currently, temporal artery biopsy is the criterion standard for the diagnosis of temporal arteritis (giant cell arteritis). A negative biopsy finding does not exclude the diagnosis. Angiography can be used when biopsy results are negative, or it can be used to help guide biopsy by demonstrating areas of abnormality.^10,11 When performed, angiography is typically directed at the large branch vessels of the proximal aorta and extracranial carotid branch vessels. The temporal arteries are depicted well in almost 90% of patients. In patients with proximal artery stenoses, angioplasty can be used in addition to corticosteroid therapy for symptomatic relief.¹²

Although angiography is one of the best-studied techniques, it is invasive and inconvenient. As a result, less-invasive procedures for evaluating the arterial anatomy have been sought. Magnetic resonance angiography (MRA) has results comparable to those of angiography in evaluating medium-to-large vessels. In some reported cases, MRA has successfully depicted disease in the temporal arteries. As the sensitivity of MRA continues to improve, it will likely become a more realistic method for evaluating stenotic lesions attributed to temporal arteritis.^13,14,15

Studies have revealed the benefit of ultrasonography in the diagnosis of temporal arteritis.^16,17,18,19 Characteristic changes, including stenoses and occlusions of temporal artery segments and a dark halo around the vessel, have been reliably observed in patients with temporal arteritis. Doppler flow studies have also been performed, with promising results.²⁰

A study of positron emission tomography (PET) scanning evaluated¹⁸ F-glucose uptake and demonstrated a sensitivity of 56%, a specificity of 98%, and a positive predictive value of 93% for the diagnosis of giant cell arteritis or polymyalgia rheumatica.²¹

Limitations of Techniques

Angiography is an excellent study; however, it is limited for temporal arteritis by the invasive nature of the examination and by the risks associated with the administration of contrast material.

Magnetic resonance angiography is a noninvasive examination, but it has limited use in evaluating smaller vessels, and imaging artifacts may result in false-positive results. In addition, larger vessels with mildly thickened walls can be missed.

Ultrasonography may not depict minor vascular changes or diseased vessels, such as intrathoracic vessels, that are not amenable to ultrasonography.

Differential Diagnoses

Arteritis, Giant Cell
Arteritis, Takayasu
Fibrous Dysplasia

Other Problems to Be Considered

Atherosclerotic disease affects the temporal arteries in rare cases, and its changes may resemble those associated with temporal arteritis.

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