Temporal Arteritis in Emergency Medicine 

  • Author: Christopher H Lee, MD; Chief Editor: Rick Kulkarni, MD   more...
 
Updated: Apr 15, 2011
 

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. See the images below.

Hematoxylin and eosin stained femoral artery brancHematoxylin and eosin stained femoral artery branch, cross section, taken from a lower limb amputation specimen. 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. Lumbar angiogram showing stenosis and occlusion ofLumbar angiogram showing stenosis and occlusion of femoral artery branches due to vasculitis in the same patient whose temporal artery biopsy specimen is shown in the previous image.

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.

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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.

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Epidemiology

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.

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Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Christopher H Lee, MD  Clinical Instructor, Section of EMS, Department of Emergency Medicine, Yale University School of Medicine

Christopher H Lee, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Jean Marie Hammel, MD  Assistant Professor, Associate Residency Director of Emergency Medicine Residency Program, Department of Surgery, Section of Emergency Medicine, Yale University School of Medicine

Jean Marie Hammel, MD is a member of the following medical societies: Alpha Omega Alpha and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard S Krause, MD  Senior Clinical Faculty/Clinical Assistant Professor, Department of Emergency Medicine, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

Richard S Krause, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: eMedicine Salary Employment

Gino A Farina, MD, FACEP, FAAEM  Associate Professor of Clinical Emergency Medicine, Albert Einstein College of Medicine; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center

Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Rick Kulkarni, MD 

Rick Kulkarni, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: WebMD Salary Employment

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Hematoxylin and eosin stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of giant cell arteritis shown here include intimal thickening with luminal stenosis, mononuclear inflammatory cell infiltrate with media invasion and necrosis, and giant cell formation in the media.
Lumbar angiogram showing stenosis and occlusion of femoral artery branches due to vasculitis in the same patient whose temporal artery biopsy specimen is shown in the previous image.
Hematoxylin and eosin stained femoral artery branch, cross section, taken from a lower limb amputation specimen. 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.
 
 
 
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