The Clinical Syndrome
As the name suggests, headache associated with temporal arteritis is located primarily in the temples, with secondary pain often located in the frontal and occipital regions. A disease of the sixth decade and beyond, temporal arteritis affects whites almost exclusively, and there is a 3:1 female gender predominance. Temporal arteritis is also known as giant cell arteritis because of the finding of giant multinucleated cells that infiltrate arteries containing elastin, including the temporal, ophthalmic, and external carotid arteries ( Fig. 12.1A ). Approximately half of patients with temporal arteritis also have polymyalgia rheumatica.
Signs and Symptoms
Headache is seen in most patients with temporal arteritis. The headache is in the temples and is usually continuous. The character of the headache pain associated with temporal arteritis is aching and has a mild to moderate level of intensity. A patient with temporal arteritis also may complain of soreness of the scalp, making the combing of hair or resting the head on a firm pillow extremely uncomfortable.
Although temporal headache is present in almost all patients with temporal arteritis, the finding of intermittent jaw claudication is pathognomonic for the disease (see Fig. 12.1B ). In an elderly patient, jaw pain while chewing should be considered secondary to temporal arteritis until proved otherwise. In the presence of strong clinical suspicion that the patient has temporal arteritis, immediate treatment with corticosteroids is indicated (see discussion of treatment). The reason immediate treatment is needed is the potential for sudden painless deterioration of vision in one eye secondary to ischemia of the optic nerve.
In addition to the signs and symptoms mentioned previously, patients with temporal arteritis experience myalgia and morning stiffness. Muscle weakness associated with inflammatory muscle disease and many other collagen-vascular diseases is absent in temporal arteritis, unless the patient has been treated with prolonged doses of corticosteroids for other systemic disease, such as polymyalgia rheumatica. The patient also may experience nonspecific systemic symptoms, including malaise, weight loss, night sweats, and depression.
On physical examination, a swollen, indurated, nodular temporal artery is present. Diminished pulses are often noted, as is tenderness to palpation. Scalp tenderness to palpation is often seen. Funduscopic examination may reveal a pale, edematous optic disc. The patient with temporal arteritis often appears chronically ill, depressed, or both.
Testing
Erythrocyte sedimentation rate should be obtained in all patients suspected to have temporal arteritis. In temporal arteritis, the erythrocyte sedimentation rate is greater than 50 mm/h in more than 90% of patients. Less than 2% of patients with biopsy-proved temporal arteritis have normal erythrocyte sedimentation rates. Ideally, the blood for the erythrocyte sedimentation rate should be obtained before beginning corticosteroid therapy because the initial level of elevation of this test is useful not only to help diagnose the disease but also as a mechanism to establish the efficacy of therapy. The erythrocyte sedimentation rate is a nonspecific test, and other diseases that may manifest clinically in a manner similar to temporal arteritis, such as malignancy or infection, also may markedly elevate the erythrocyte sedimentation rate. Confirmation of the clinical diagnosis of temporal arteritis requires ultrasound imaging and/or a temporal artery biopsy. Ultrasound imaging can provide confirmation by identification of a halo sign surrounding the affected temporal artery. The finding of a positive halo sign strongly supports the diagnosis of temporal arteritis ( Fig. 12.2 ).
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