Temporal Arteritis

Clinical Features

Temporal arteritis is the local (and most frequent) manifestation (in temporal artery) of giant cell arteritis (GCA). Polymyalgia rheumatica (PMR), although separately described initially, is known to be the same fundamental process. Involvement of the retinal artery with blindness is a dreaded complication. Elevated erythrocyte sedimentation rate (ESR) is a regular laboratory feature. Adrenal glucocorticoids are effective therapy. Temporal arteritis was first described by Jonathan Hutchinson (1890) in 'an old man named Rumbold, the father of a well-remembered beadle at the London Hospital College....He was...quite bald....he had had red 'streaks on his head' which were painful and prevented his wearing his hat. The 'red streaks' proved on examination to be his temporal arteries, which on both sides were found to be inflamed and swollen. The streaks extended from the temporal region almost to the middle of the scalp, and several branches of each artery could be distinctly traced. The condition was nearly symmetrical. During the first week that he was under observation pulsation could be feebly detected in the affected vessels, but it finally subsided, and the vessels were left impervious cords.'

Foroozan et al. (2002) compared platelet counts, complete blood counts, and Westergren sedimentation rates (WESR) of biopsy-positive GCA patients with biopsy-negative patients. They found that elevated WESR was more sensitive (79%), while elevated platelet count was more specific (91%) than elevated WESR or the combination of elevated WESR and elevated platelet count. The authors concluded that in patients suspected of having GCA, an elevated platelet count greater than 400,000/mcl is a useful marker of positive temporal artery biopsy.

Pache et al. (2002) tested the hypothesis that plasma endothelin-1 (131240) would be increased in 4 patients with biopsy-proven giant cell arteritis. All patients showed significantly increased plasma levels of endothelin-1, although the clinical relevance of the increase required further evaluation.

Salvarani et al. (2002) reviewed polymyalgia rheumatica and giant cell arteritis in detail.

Hall et al. (2003) examined the role of unilateral temporal artery biopsy in suspected giant cell arteritis. In their series of 181 patients, unilateral temporal artery biopsy was associated with an extremely low frequency (1%) of subsequent positive contralateral biopsy and was not associated with adverse visual or neurologic outcomes for any subject. The authors concluded that in the hands of experienced physicians, a unilateral temporal artery biopsy is sufficient to exclude a diagnosis of GCA in populations for which clinical suspicion is low. Jaw claudication, pale optic disc edema (particularly 'chalky white' disc edema), fever, or any systemic symptom other than headache should raise suspicion of a diagnosis of GCA.

Among patients with giant cell arteritis who experienced visual deterioration, Hayreh and Zimmerman (2003) reported that further visual loss occurred in a few patients despite treatment with corticosteroids: 13% of patients treated with intravenous steroids versus 3% of patients who received oral steroids developed further visual deterioration. However, the difference between the 2 groups was not statistically significant.

Familial aggregation has been observed. Usually sibs have been affected, but mother-daughter or father-daughter pairs have been reported. Granato et al. (1981) reviewed familial occurrences of giant cell arteritis and PMR and reported father and daughter with giant cell arteritis. How et al. (1981) reported PMR developing in 2 sisters at ages 74 and 72 years, respectively. Familial aggregation in this condition is probably of the same order and same basis as that of many autoimmune disorders (109100) such as Hashimoto struma (140300), Schmidt syndrome (269200), autoimmune hemolytic anemia (205700), lupus erythematosus (152700), alopecia areata (104000), pernicious anemia (170900), etc.

Zauber et al. (1997) described temporal arteritis in a man and his daughter who presented with symptoms at ages 78 and 59 years, respectively. The father's brother had also had a clinical diagnosis of temporal arteritis.

Liu et al. (2001) reviewed 121 consecutive patients who underwent temporal artery biopsy for possible GCA. The mean age of the patients with biopsy-proven GCA was higher than that in the biopsy-negative group (75 years vs 69 years). The authors found no statistical correlation between biopsy-positive and biopsy-negative groups with respect to gender or ESR. However, when they controlled for race, the ESR was higher among white patients. Positive biopsies were found in 29% of white patients, 11% of Asian patients, and none of 40 Hispanic and 6 African American patients. The authors concluded that further studies were needed to determine the genetic factors protecting Hispanic patients against GCA.

Lam et al. (2007) performed a prospective survey of 134 of 257 patients at their institution who underwent temporal artery biopsy over a 6-year period. They found the prevalence and clinical course of GCA to be similar in the 65 Hispanic and 69 non-Hispanic patients.

From a retrospective review, Pereira et al. (2011) found that biopsy-proven giant cell arteritis was 20 times less frequently seen in Asian than in Caucasian persons in their patient population. The population served by their facility was 42% Caucasian, 28% Asian, and 30% other. The difference in the proportion of GCA in Asians and Caucasians was statistically significant (odds ratio = 0.049; CI, 0.0065-0.374; p = 0.036).

To address the question of whether gene products of the HLA class II complex might contribute to GCA, Weyand et al. (1992) analyzed the functionally most important locus, HLA-DRB1 (see 142860), in a cohort of patients with biopsy-proven disease. Among the patients, 3 allelic variants of the HLA-DRB1*04 family were found to be overrepresented. Interestingly, GCA was linked to the same allelic variant as rheumatoid arthritis (RA; 180300). However, a consecutive case series study demonstrated that GCA and RA rarely co-occurred, supporting the interpretation that distinct functional domains of the HLA-DR molecule are implicated in the pathomechanisms of these 2 autoimmune diseases. Such was supported further by the finding of a sequence motif spanning the amino acid positions 28-31 that was shared by all GCA patients and different from that found in RA patients. This sequence stretch translated into a polymorphic site in the antigen-binding groove of the HLA-DR molecule, suggesting a crucial role in the selection and presentation of antigen to T lymphocytes.