Temporal arteritis

Clinical manifestations
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By James Goodwin MD

New headache or dramatic change in pattern of longstanding headache in an elderly patient is the most common presentation of temporal arteritis. The pain may have a local component along the temporal and other external carotid branches, but often the character of the headache is nonspecific (Hamilton et al 1971). Only a few individuals have localized pain, induration, and tenderness over the temporal arteries or erythematous nodules along the vessels, though it is worth looking for these physical signs. In some cases the temporal arteries are palpably indurated and may show a "ropy" appearance beneath the skin.

The incidence of headache in temporal arteritis is about 80% in large series, so one cannot dismiss the diagnosis entirely in the absence of headache. Severity of the headache is variable, and headache was the symptom that led to referral of only 41 patients of 175 patients in the 1960 Mayo Clinic series. The typical headache, if one can be said to occur, is maximal over the temples and suboccipital region. Occipital arteries as well as branches of the external carotid artery are commonly involved, and pain in these vessels should be sought during the history and by palpation. Tenderness specifically along extracranial branches of the carotid system is particularly useful in diagnosis when present. Momentary stabbing or ice pick headache accompanying new-onset persistent headache in an elderly person may be a useful indicator of temporal arteritis as the cause (Rozen 2010).

Diffuse tenderness of the scalp, face, or oral mucosa results from ischemia secondary to widespread arteritic involvement of extracranial vessels. Some patients describe "head pains" rather than headache, indicating the relatively superficial character of the discomfort (Paulley and Hughes 1960). Patients should be asked if they are experiencing new tenderness when they comb their hair, or if a hat or the temple pieces of their glasses are causing new pain in 1 or both temples. The pain in the vessel may be dependent on pulsatile blood flow and may cease with thrombosis of the vessel. Similarly, the pain may be relieved when an involved segment of temporal artery is removed for biopsy. When present, pain relief after biopsy is a strong element favoring the diagnosis, and patients should be asked about this a few days after the biopsy when the local pain from the surgery has subsided.

Pain and ulceration of the oral mucosa tongue or scalp is an especially useful sign favoring temporal arteritis. Scalp necrosis and ulceration can be mistaken for herpes zoster. The histopathology in temporal arteritis consists of lymphocytic vasculitis, scarring with myxoid degeneration, and fat necrosis in the dermis (Ghaffar and Todd 2010). Ischemia of the tongue can be misdiagnosed as glossitis with beefy enlargement and loss of taste sensation. In a review, the mean interval between onset of temporal arteritis and scalp necrosis was 3 months in 19 patients who had not been started on corticosteroids.

Jaw claudication is nearly pathognomonic for temporal arteritis, though it can be present on the basis of severe atherosclerosis of the external and internal carotid arteries in rare instances. Both the internal and external carotid systems must be affected together to produce jaw claudication, as each system provides collateral flow to the other when occlusive disease involves only 1. Jaw claudication must be distinguished from other causes of pain around the lower face and dentition. The pain of claudication builds progressively with the muscular activity of chewing rather than occurring with the pressure of biting down, whereas dental pain occurs even with the first bite and pain occurs with any jaw movement with pathology in the temporomandibular joint. As with claudication elsewhere, the pain is relieved with rest and usually requires at least a few seconds to dissipate when the patient stops chewing. Careful history taking is the only way to elicit these fine points and correctly identify claudication or ischemia as the mechanism of pain.

Many patients with temporal arteritis have low-grade fever, leukocytosis, or mild anemia either at presentation or during the course of the illness. Depression, listlessness, and general malaise are common complaints among the elderly, but increased intensity of 1 or more of these symptoms may signal the onset of temporal arteritis. Nonspecific complaints like these have been shown to precede the more specific symptoms, even for several months, in up to half the patients. Weight loss, weakness, and inanition may be profound in the occasional patient.

Isolated psychiatric symptoms with psychotic features can be a presenting feature of temporal arteritis. Sudden onset of psychosis in the elderly may warrant a short course of corticosteroids. Prompt remission of symptoms with steroid treatment is another important factor in diagnosis.

Low back pain can be the presenting symptom of abdominal aortic giant cell arteritis, and 18 FDG-PET is useful to identify the aortic involvement in this setting (Fernandez-Lopez et al 2007).

Occasionally, ischemic complications can appear before any of the systemic signs or symptoms of illness, making diagnosis extremely difficult. Some authors have called this type of presentation "occult temporal arteritis" (Cullen 1963).

Although the signs and symptoms of temporal arteritis are variable, once they have begun they are usually continuous or progressive until effective treatment is established, but Purvin and Kawasaki described 4 cases with spontaneous remission of symptoms and signs (Purvin and Kawasaki 2007).

Because of its greater-than-chance association with temporal arteritis, it is important to be aware of the signs and symptoms of polymyalgia rheumatica. It is not uncommon for patients with polymyalgia to develop additional symptoms of temporal arteritis after they have had the original illness for some time, sometimes years.

Polymyalgia, like temporal arteritis, almost exclusively affects persons over the age of 50 years. The clinical picture consists of pain and stiffness in the shoulder or pelvic girdles. Pain and limited motion of the axial skeleton including the cervical and thoracic spine are common. Pain and stiffness are often worse on arising in the morning, with some improvement of mobility as the day proceeds. Onset may be insidious or acute. Severity of symptoms can be asymmetric, but the disorder is invariably bilateral. There may be mild swelling of the shoulders and even distal joints in the extremities, but as a rule no articular deformity is manifest either clinically or by radiographic examination.

Pain is almost always accompanied by systemic symptoms identical to those encountered in temporal arteritis. Headache, jaw claudication, and scalp pain or tenderness define the clinical transition from polymyalgia to temporal arteritis and determine the need for more aggressive corticosteroid or immunosuppressive management. Axillary artery bruit can be a presenting sign of transition to giant cell arteritis in patients with polymyalgia rheumatica (Czihal et al 2011). The authors point out that axillary artery bruit is very uncommon in atherosclerosis.

On physical examination there are few findings in patients with polymyalgia. One prominent characteristic feature is that the passive range of motion in neck or pelvis far exceeds the active range. That is, the examiner can slowly move the patient's neck through a greater range than the patient can by using his or her own neck muscles. Patients with polymyalgia have an increased incidence of arterial bruits over the large arteries of the arms and legs as well as over the carotid and subclavian vessels.

Polymyalgia rheumatica may have a range of atypical presentations including peripheral synovitis, mild weakness, and normal sedimentation rate. In fact, there is considerable overlap between polymyalgia rheumatica and seronegative rheumatoid.

The duration of polymyalgia has ranged in various reports from 6 months to as long as 14 years, but most patients are ill with it for 1 to 3 years.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited