The most common systemic vasculitis in adults is temporal arteritis. Its classic manifestations—headache, polymyalgia rheumatica, jaw claudication, and vision changes—are well known to most physicians. More than 70 percent of patients complain of headache (although perhaps not as the initial symptom); however, the headache is often described as being “different” from a patient's usual headache or as a new-onset headache. Some patients even develop an occipital headache caused by inflammation of the occipital artery. Hellmann reviewed a case report showing atypical symptoms of temporal arteritis.
Pain in the facial muscles caused by chewing (jaw claudication) occurs in many patients with temporal arteritis, although only one half of them have typical jaw claudication. Some complain of a nonspecific dental discomfort or a pain in the sinus or ear region. Polymyalgia rheumatica (PMR), which is closely linked to temporal arteritis, presents with pain and weakness in the hip girdle, shoulders, and neck, with pain being the predominant complaint. Patients with PMR may have difficulty getting out of bed. Other common findings include an erythrocyte sedimentation rate (ESR) greater than 50 mm per Hg and, in one half of patients, an abnormal temporal artery on physical examination.
The American College of Rheumatology's basis for temporal arteritis includes the presence of three of the following criteria: (1) age 50 years or older, (2) new-onset headache, (3) temporal artery tenderness, (4) ESR of at least 50 mm per Hg, and (5) abnormal temporal artery biopsy. However, these are not diagnostic criteria, and atypical presentations are not uncommon.
Hellmann maintains that temporal arteritis should be suspected in patients older than 50 years who have unexplained pain above the neck, because temporal arteritis rarely occurs in patients younger than 50 years (the average age of presentation is 72 years) and because an atypical presentation is possible. In addition to the classic presentations of temporal arteritis, Hellmann discusses some of the atypical presentations of this panarteritis that preferentially involves the extracranial carotid arteries. The accompanying table lists some of the atypical manifestations.
The most worrisome complication of temporal arteritis is blindness caused by anterior ischemic optic neuropathy. This feature develops, on average, five months after the initial symptoms of temporal arteritis. Because visual symptoms are rarely the presenting symptoms, blindness can usually be prevented if the other presentations of this condition are recognized. Approximately 10 percent of patients have respiratory symptoms (e.g., dry cough, sore throat, tongue pain, hoarseness, a choking sensation), and these symptoms are the presenting complaint in about 4 percent of patients. However, a dry cough is never the only symptom in a patient with temporal arteritis. A respiratory work-up, including chest radiography, will be normal in patients with respiratory symptoms and temporal arteritis. Another finding is fever of unknown origin. Patients with temporal arteritis and fever of unknown origin have normal white blood cell counts. It is not clear why some patients with temporal arteritis develop certain sets of symptoms.
Diagnosis almost always requires unilateral temporal artery biopsy. The results of this procedure can be accurate if the patient has been taking corticosteroids for less than two weeks. Prednisone therapy should not be delayed if temporal arteritis is suspected. However, it may be difficult to taper prednisone (because of relapses), and the patient may need to continue treatment for more than two years.