Introduction
Background
Temporal arteritis (TA), also known as giant cell arteritis (GCA), is a common form of systemic vasculopathy affecting patients older than 50 years. Although typically affecting the superficial temporal arteries, this inflammatory process has been shown to involve medium- and large-sized vessels, including the aorta, carotid, subclavian, vertebral, and iliac arteries. Therefore, "giant cell arteritis" may be more appropriate than "temporal arteritis" to identify this type of vasculitis, though both terms are used interchangeably.
Hematoxylin and eosin stained femoral artery branch, cross section, taken from a lower limb amputation specimen from the same patient shown in Media files 1-2. Mononuclear cell invasion and necrosis in the media of this large artery can be observed. Extensive lower limb vasculitis from giant cell arteritis resulted in ischemic necrosis of the lower limb, necessitating amputation.
The most devastating complication of temporal arteritis is irreversible vision loss, which was first described in 1937. Currently, temporal arteritis is considered one of the most important ophthalmic emergencies as bilateral blindness can occur in up to one third of patients. For emergency physicians, early recognition and prompt treatment are critical to prevent permanent ischemic damage to the retina and optic nerve.
Pathophysiology
The exact etiology of this disease remains unknown. Temporal arteritis is a chronic, systemic vasculitis primarily affecting the elastic lamina of medium- and large-sized arteries. Histopathology of affected arteries is marked by transmural inflammation of the intima, media, and adventitia, as well as patchy infiltration by lymphocytes, macrophages, and multinucleated giant cells. Mural hyperplasia can result in arterial luminal narrowing, resulting in subsequent distal ischemia.
The temporal artery is commonly affected, often resulting in temporal-lobe headaches. Other commonly affected vessels include the ophthalmic, posterior ciliary, and, to a lesser extent, the central retinal artery. Inflammation in these locations can cause irreversible visual impairment and ischemic optic neuritis.
Despite increased understanding of the inflammatory cascade responsible for the disease process, the initial event that triggers the cascade remains uncertain. Although many infectious pathogens, such as Parvovirus B19 and Chlamydia species , have been suggested as possible inciting agents, the actual role of microbial pathogens is still unclear.
Current theory regarding the etiology of temporal arteritis holds that a maladaptive response to endothelial injury leads to an inappropriate activation of cell-mediated immunity via immature antigen-presenting cells. The subsequent release of cytokines within the arterial vessel wall can attract macrophages and multinucleated giant cells, which gives diseased vessels their characteristic histology. This also leads to an oligoclonal expansion of T-cells directed against antigens in or near the elastic lamina. Ultimately, this cascade results in vessel wall damage, intimal hyperplasia, and eventual stenotic occlusion.
These inflammatory changes are also seen in polymyalgia rheumatica (PMR). Polymyalgia rheumatica and temporal arteritis are closely related inflammatory conditions, and it is suggested that they may be slightly different manifestations of the same underlying disease process. The symptoms of polymyalgia rheumatica are more systemic, including pain and stiffness in the shoulder and pelvic musculature, as well as fever, malaise, and weight loss. The relationship between polymyalgia rheumatica and temporal arteritis warrants consideration as it has been estimated that approximately half of patients initially presenting with temporal arteritis have been found to also have polymyalgia rheumatica. Conversely, about 10% of patients initially presenting with polymyalgia rheumatica were found to have temporal arteritis upon further investigation.
The etiology of temporal arteritis is multifactorial, as both genetic and environmental associations have been identified. Some major histocompatibility complex molecules, particularly human leukocyte antigen HLA-DR4 and HLA-DRB104 alleles, may have a role in a patient’s susceptibility to temporal arteritis. Additionally, there is a statistically significant increase in the incidence of temporal arteritis in northern latitudes.
Frequency
United States
Temporal arteritis is typically seen in female patients older than 50 years. Incidence increases with age and can range from 1 in 10,000 to 5 in 10,000.
International
Rates are significantly higher in northern latitudes.
Mortality/Morbidity
Temporal arteritis does not appear to affect long-term survival.
Permanent visual loss is the most devastating consequence of temporal arteritis. The incidence of ocular involvement varies greatly in the literature, ranging from 8-50%. Bilateral visual loss can occur in up to 33% of patients.
Rarely, patients can experience neurologic manifestations such as transient ischemic attacks (TIAs) or cerebral vascular accidents (CVAs)1 . However, the exact relationship between temporal arteritis and TIA/CVA is uncertain.
Race
Temporal arteritis occurs more frequently in white patients, especially those of northern European descent.
Sex
Women develop temporal arteritis 2-3 times more frequently than men.
Age
Temporal arteritis rarely occurs in patients younger than 50 years. The mean age of onset is 72 years. Incidence of the disease increases significantly with increasing age.
Clinical
History
Headache is the most common chief complaint and presents in over two thirds of patients with temporal arteritis. The headache tends to be new or different in character than previous headaches and is typically sudden in onset, localizing to the temporal region. However, pain with temporal arteritis can occur diffusely through the occipital, frontal, or parietal regions as well. Therefore, any new headache in patients older than 50 years warrants a consideration of temporal arteritis.
Because temporal arteritis tends to affect the branches of the carotid artery, clinical manifestations vary depending on the distribution of the ischemic vessel. For example, superficial temporal artery involvement can lead to severe scalp tenderness during such simple acts as resting the head on a pillow, combing hair, or wearing hats and eyeglasses. Patients may also present with visible areas of scalp necrosis. Similarly, jaw claudication while speaking or chewing is observed in patients with involvement of the maxillary artery, which can occur in half of patients with temporal arteritis.
Visual loss may also be a presenting symptom and can be sudden and painless. Initial visual symptoms are usually transient and intermittent, typically manifesting as unilateral visual loss or occasionally diplopia. However, if left untreated, permanent blindness frequently results.
Constitutional symptoms due to systemic inflammation are common. These nonspecific symptoms include fever, malaise, memory impairment, anorexia, weight loss, fatigue, and depression. Additionally, polymyalgia rheumatica symptoms are present in about half of all cases of temporal arteritis and may be the initial complaint in many patients.
Based on the 1990 American College of Rheumatology criteria for classification of temporal arteritis, at least 3 of the following 5 items must be present (sensitivity 93.5%, specificity 91.2%)2 :
- Age of onset older than 50 years
- New-onset headache or localized head pain
- Temporal artery tenderness to palpation or reduced pulsation
- Erythrocyte sedimentation rate (ESR) greater than 50 mm/h
- Abnormal arterial biopsy (necrotizing vasculitis with granulomatous proliferation and infiltration)
Physical
A thorough physical and neurological examination should be performed to exclude other possible causes of headache and visual disturbances.
The head and face should be examined for inflamed and thickened arteries, tenderness to palpation, tender scalp nodules, or necrotic areas of the scalp. Inflamed vessels may be tender and warm. They may also appear thickened and dilated, such that the examiner may be able to roll the artery between the fingers and the skull. Cranial nerve palsies, particularly of the sixth nerve, should also be noted.
A complete eye examination should be performed, including visual acuity, visual field check, and funduscopic as well as a slit lamp examinations. Special attention should be paid to the retinal vessels, as other causes of loss of vision such as central retinal artery or vein occlusion can cause a markedly abnormal funduscopic examination result. In temporal arteritis, the funduscopic examination result may be normal or can show dilated retinal veins.
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| Multimedia: Temporal Arteritis |
| References |
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Further Reading
Keywords
TA, temporal arteritis, GCA, giant cell arteritis, cranial arteritis, vasculitis, systemic vasculopathy, temporal-located headaches, ischemic optic neuritis, headache, cephalgia, impaired vision, vision loss, jaw claudication, sixth nerve palsy, afferent pupillary defect, temporalarteritis, ophthalmic emergency, blindness, arteritic ischemic optic neuropathy
