6.5: Arteritis

The most common type of arteritis seen by the neurologist, ophthalmologist, or neuro-ophthalmolgist is temporal arteritis. The initial manifestation of the disorder is headache in up to 70 percent. It is a significant cause of headache in the elderly population. Eighteen of nineteen patients with biopsy-proven giant cell arteritis evaluated by Hauser et al. (1971) initially presented with temporal pain or headache. Of the 100 patients with giant cell arteritis examined by Calamia and Hunder (1980), headaches occurred at some time in 68 and as the initial manifestation of the disease in 32. On the other hand, in the series reported by Healey and Wilske (1978), only 3 of 50 patients with giant cell arteritis complained of headache or neck pain. Nevertheless, Goodman (1979), in a major review of the literature on giant cell arteritis, puts the prevalence of headache around 60%, while Hamilton et al. (1971) and Eshaghian (1979) quote a figure of 50%. The headache may be severe and ``boring'' in nature and may be specifically associated with tenderness overlying the superficial temporal arteries. We have seen several patients who were unable to comb or brush their hair or sleep on one side because of severe temporal tenderness. The headache may be so severe as to be unrelieved by barbiturates or by morphine. The pain may radiate to the neck, face, jaw, or tongue. Ear pain and jaw claudication may also be initial symptoms of this disease. The temporal arteries may be palpable and are enlarged during the period of headache. In some instances, patients with giant cell arteritis may appear chronically ill. They may have anorexia, weight loss, fever, myalgias, diplopia, and/or blindness from central retinal artery occlusion, anterior or retrobulbar ischemic optic neuropathy, or cerebral ischemia. They may be found to have anemia, an elevated white blood cell count, and--in most cases--an elevated erythrocyte sedimentation rate.

A majority of the patients affected by giant cell arteritis are women (Hoyt et al., 1941; Goodman, 1979; Calamia and Hunder, 1980), and almost all patients are over 50 years of age, with an average age in the mid-70's (Hoyt et al., 1941; Huston et al., 1978; Goodman, 1979). Although patients under 50 years of age have been identified, they remain quite rare. According to Hauser et al. (1971), the prevalence of giant cell arteritis is 33/100,000 in the age group from 60---69 years of age and 844/100,000 in patients over 80 years of age.

The general lesion is one of chronic periarteritis and arteritis, with areas of granulation tissue in the adventitia of the vessels, as well as round cell infiltration in the same region. Reinecke and Kuwabara (1969) thought that degeneration of smooth muscle cells in the media was the initial pathologic change, followed by secondary fragmentation of the elastic lamina and giant cell reaction. These investigators identified three stages of disease: (1) swelling and degeneration of muscle cells surrounded by inflammatory cells; (2) disappearance of muscle cells with replacement by connective tissue and inflammatory cells including lymphocytes and multinucleated giant cells; fragmentation of elastic fibers; and (3) disappearance of inflammatory cells, with fibrosis of the media and some regeneration of muscle cells and elastic fibers.

Ricker et al. (1979), in a prospective electron microscopic study of positive temporal artery biopsies, found evidence of extensive smooth muscle degeneration of the arterial wall with active phagocytosis of the degenerated smooth muscle fragments. Thus, the smooth muscles of the arteries appear to be the primary site of pathology. Studies by Hazelman et al. (1975) suggest that arterial antigen-stimulated lymphocytes are present in patients with active giant cell arteritis prior to, but not after, treatment. It would appear that elastin may provoke a cell-mediated immune response.
Because of the severe systemic complications that occur in patients with giant cell arteritis, particularly severe visual loss that occurs in 40---50% of patients (Hollenhorst et al., 1960; Spencer and Hoyt, 1960), it is imperative that this disease be diagnosed as early as possible. Zanen et al. (1951) reported an individual who, after having had severe headaches for 3 weeks, developed bilateral visual loss, optic disc swelling, and retinal hemorrhages first in one eye and then in the other. A temporal artery biopsy confirmed the diagnosis. Thus, the disease should be considered in all elderly patients complaining of headache, whether or not visual symptoms are present. If the disease is diagnosed and treated early with high dose systemic corticosteroids, the severe visual, intracranial, renal, and cardiac complications may be prevented. Unfortunately, we continue to see many patients in whom giant cell arteritis is diagnosed only after visual loss occurs, despite their having had significant systemic symptoms, particularly headache, weeks to months before visual loss.

Temporal arteritis or giant cell arteritis is a systemic disorder of unknown cause characterized by an inflammatory obliterative arteritis particularly, but not exclusively involving branches of the external carotid and ophthalmic arteries. It is well known as a cause of anterior ischemic optic neuropathy and less well recognized as a cause of ophthalmoplegia. The most common initial symptom is headache, often accompanied by diffuse aches and pains (polymyalgia rheumatica). (Allen and Studenski, 1986) Other common symptoms include jaw claudication, fever, anemia, and weight loss. In the study of Vilaseca et al., (1987) the simultaneous presence of recent onset headache, jaw claudication and abnormalities of the temporal arteries on physical examination had a specificity of 94.8% compared to the histological diagnosis and 100% with respect to the final diagnosis.

Solomon and Cappa (1987) point out that the headache of temporal arteritis may clearly involve more than the temporal area and include pain in the temporal, frontal, vertex and occipital regions. The headache is characterized by gradual onset, progressing to a diffuse, often severe, aching. The headache may be intermittent, but usually becomes a prominent, if not daily, feature of patients with the disorder. The headache usually is constant and perceived as superficial in the scalp. There may be exquisite tenderness of the scalp and blood vessels particularly in the temporal region. The headache is usually worse at night and may be especially aggravated by exposure to cold (Raskin, 1988).

Sixty-five percent of patients are women with the average age at onset of 70 (range 50-85). It is a common disorder and must be actively sought in any headache patient presenting after the age of 50 particularly in those with systemic symptoms. Allen and Studenski (1986) emphasize additional symptoms such as extremity and tongue claudication, ear pain, stroke, and angina as well as systemic panarteritis involving the peripheral nervous system and abdominal or pelvic viscera. Up to 15 percent of patients with temporal arteritis may have angiographic evidence of extracranial giant cell arteritis. Aortic insufficiency, ruptured aortic aneurysm, aortic dissection, stroke, or myocardial infarction may be the initial manifestation of systemic giant cell arteritis (Lie, 1995).

Other ocular complaints with temporal arteritis include tonic pupils from ischemia of the ciliary ganglia (Currie and Lessel, 1984) and bilateral uveitic glaucoma which may be on an immunologic basis (Copetto et al, 1985). While most attention has been drawn to vascular complications in the distribution of the external carotid and ophthalmic arteries, rarely patients present with vertebral basilar symptomatology (Monteiro et al, 1984). Among the protean manifestations of temporal arteritis are those described in the following reports: bilateral carotid artery occlusion (Howard et al, 1984), renal disease (Truong et al, 1985), aortic arch arteritis (Perruquet et al, 1986), temporal mandibular joint pain (Selsky and Nirankari, 1985), painful facial swelling (Accetta et al, 1985), jaw claudication (Goodman and Shepard, 1983), and sudden death from arteritis in the coronary arteries, dissection of the aorta and major cerebrovascular accident (Save-Soderbergh et al, 1986).

An elevated sedimentation rate has been considered by some indispensable in diagnosing temporal arteritis. Without an elevated sedimentation rate, even in patients with a classic history and clinical findings, the diagnosis might be abandoned without proceeding to a temporal artery biopsy. Nonetheless, it is now estimated that up to 9% of patients with temporal arteritis may have normal sedimentation rates (Wong and Korn, 1986; Villalta and Estrach, 1985). Wong and Korn (1986) have identified 37 reported cases of biopsy-proven temporal arteritis with a normal Westergren sedimentation rate (< 40 mm/hr in patients > than 50 years old).

Up to one third of temporal artery biopsies may be falsely negative, especially when one fails to examine a long segment of vessel by serial sections. As recently stated, "because of the danger to vision, most authorities would start steroid treatment when they suspect the diagnosis clinically. Any response other than prompt and striking improvement in clinical well-being and symptoms, however, speaks against the diagnosis." (Plum, 1986) Raskin (1988) and Allen and Studenski (1986), and others believe steroid therapy should start immediately, before confirmation by laboratory and pathologic determinations. They suggest in very ill patients, intravenous methylprednisolone may be better. Fluorescein angiography may be helpful in the diagnosis of giant cell arteritis (Siatkowski, et al, 1993). Patients in this study had higher erythrocyte sedimentation rates, larger cup/disk ratios, and delayed fluorescein dye appearance and choroidal filling times. Interestingly enough, biopsy positive patients with temporal arteritis have an increased incidence of headache (93 percent versus 62 percent), more jaw claudication (50 percent versus 18 percent), and prior temporomentology suggesting polymalgia rheumatica (23 percent versus 3 percent) in a study of almost 100 patients (Chmelewski et al, 1992).

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