eMedicine Specialties > Rheumatology > Vasculitis

Giant Cell Arteritis

Author: Mythili Seetharaman, MD, Consulting Staff, Einstein Arthritis Center, Albert Einstein Medical Center, St Christopher's Hospital for Children
Coauthor(s): Stephen A Paget, MD, Chair, Division of Rheumatology, Chief, Department of Medicine, Hospital for Special Surgery; Professor, Department of Internal Medicine, Division of Rheumatology, Weill-Cornell University; Evan Leibowitz, MD, Fellow, Department of Internal Medicine, Division of Rheumatology, Valley Hospital
Contributor Information and Disclosures

Updated: Nov 14, 2008

Introduction

Background

Giant cell arteritis (GCA), also known as temporal arteritis or cranial arteritis (coined by Horton et al,1 ) is a systemic, inflammatory, vascular syndrome that predominantly affects the temporal arteries. Giant cell arteritis is commonly associated with polymyalgia rheumatica (PMR). About half of persons with giant cell arteritis have underlying polymyalgia rheumatica, whereas about 15% of individuals with polymyalgia rheumatica develop giant cell arteritis.

In 1957, Barber described polymyalgia rheumatica as an aching syndrome that was not associated with other defined rheumatic, infectious, or neoplastic disorders and that usually occurred in elderly persons with constitutional symptoms and an elevated erythrocyte sedimentation rate (ESR).2

Pathophysiology

The etiology of giant cell arteritis is unknown, but the pathogenesis involves a chronic inflammatory process, predominantly of large arteries, resulting in the elaboration of various cytokines.

The specific cytokine production by the involved tissues may influence how a disease presents clinically. Cytokine profiles for giant cell arteritis and polymyalgia rheumatica are different. In giant cell arteritis, inflamed temporal arteries contain the T-lymphocyte products interferon-gamma (IFN-gamma) and interleukin (IL)–2. Giant cell arteritis also contains the macrophage products IL-1-beta, IL-6, and transforming growth factor-beta (TGF-beta). In polymyalgia rheumatica, temporal arteries contain transcripts for TGF-beta, IL-1, and IL-2 but not IFN-gamma.

Patients with giant cell arteritis, who present with fever of unknown origin and who do not have ischemic symptoms, typically have low IFN-gamma levels. Arteries that express high IFN-gamma levels typically have multinucleated giant cells (MGCs). Unlike macrophages, MGCs have important secretory functions and do not only remove debris. MGCs secrete platelet-derived growth factor (PDGF), which stimulates intimal hyperplasia. MGCs also secrete vascular endothelial growth factor (VEGF), a critical mediator of angiogenesis in the arterial wall.

Researchers recently described the topography of inflammatory cells in different regions of the artery, as follows:

  • Adventitial macrophages produce IL-1 and IL-6.
  • Medial macrophages produce metalloproteinase-2, PDGF, and VEGF.
  • Intimal macrophages produce nitric oxide synthetase-2 and TGF-beta.
  • Adventitial T lymphocytes produce IFN-gamma.
  • T lymphocytes may enter the arterial wall through the vasa vasorum, which exist in the adventitia.
  • The adventitia is the likely site of initial immunologic injury and is considered the immunological center in the pathogenesis of giant cell arteritis.

Concentric intimal hyperplasia is an important underlying pathologic lesion in giant cell arteritis. Researchers assume that intimal hyperplasia occurs when the blood vessel wall responds to injury and that it is a repair mechanism. PDGF is important in stimulating intimal hyperplasia. PDGF derives from macrophages and giant cells, distinguishing giant cell arteritis from other vasculopathies. For example, resident smooth muscle cells, rather than monocytes, produce most of the PDGF in atherosclerotic disease. In giant cell arteritis, the media is the main site of injury. The medial macrophages release the tissue-destroying enzymes and mediate tissue repair by secreting factors (eg, PDGF, VGEF), resulting in a hyperplastic intima that obstructs the blood vessel lumen.

The intima and media layers are the histological center in the pathogenesis of giant cell arteritis. Cell adhesion molecules influence the pathogenesis, and endothelial cells play a pivotal role. Inflammation is an important process that influences the endothelium and causes neovascularization. This process occurs mainly at the intima-media junction and at the adventitial layer. Adhesion molecules are far more intensely expressed on these neovessels than in the vessel lumen. Using immunochemical staining, Cid et al (2000) demonstrated that different adhesion molecules might regulate how leukocytes and endothelial cells interact in different temporal artery layers.3

Systemic manifestation is likely related to the inflammatory process and cytokine elaboration, while end organ involvement is related to vascular occlusion.

Frequency

United States

Polymyalgia rheumatica affects approximately 1 per 1000 persons older than 50 years. The true incidence of giant cell arteritis is unknown, but researchers suggest the rate is 33% that of polymyalgia rheumatica. In the United States, the approximate incidence of giant cell arteritis ranges from 0.49-27.3 cases per 100,000 population among persons older than 50 years.4 Salvarani et al (1995) found that the incidence of giant cell arteritis in Olmsted County, Minnesota, was about 17.8 per 100,000 persons older than 50 years.5

International

The incidence of giant cell arteritis is increased in northern latitudes, especially in Scandinavian countries. Smeeth et al (2006) found that the incidence of giant cell arteritis in the United Kingdom from 1990-2001 was 2.2 cases per 10,000 person-years.6 Giant cell arteritis is much less common in Saudi Arabia than in Europe or the United States.7

Mortality/Morbidity

  • The morbidity of polymyalgia rheumatica relates to the treatment complications associated with long-term steroid therapy (eg, osteoporosis, hypertension, diabetes, cataract formation, increased infection risk). Patients with giant cell arteritis also experience steroid-related complications; however, they are also at risk for blindness. Ischemic optic retinopathy develops in approximately 26% of patients with giant cell arteritis, leading to permanent visual loss in approximately 7%-14%.8
  • In very rare cases, giant cell arteritis involves the CNS, producing seizures, cerebral vascular accidents, or abnormal mental status. This is despite the fact that giant cell arteritis, for the most part, occurs only in vessels with an elastica, a finding that is not present with intradural blood vessels. However, involvement of the aortic arch vessels, including the subclavian arteries, can lead to subclavian steal syndrome and brain ischemia. Rarely, intracranial vessels are involved. The frequency of giant cell arteritis leading to significant ischemic CNS disease is not known because the intracranial vessels are relatively inaccessible and arteriosclerotic vascular disease is prevalent in older patients. Peripheral nerve involvement is also rare.
  • Studies show that giant cell arteritis is a basis for aneurysms, dissections, stenotic lesions of the aorta, and stenotic lesions of the major branches of the aorta. Isolated cases of coronary artery disease and various aortic arch syndromes are evident secondary to giant cell arteritis. Involvement of the abdominal aorta, similar to that of the thoracic aorta, can produce symptoms secondary to aortic aneurysms and intestinal infarction. Some clear-cut examples of arm or leg claudication are reported secondary to giant cell arteritis.

Race

  • Both polymyalgia rheumatica and giant cell arteritis are much less common in African Americans than in whites. Giant cell arteritis is rare in the Hispanic population.9

Sex

  • Polymyalgia rheumatica and giant cell arteritis are twice as common in women as in men.

Age

  • Most persons with polymyalgia rheumatica or giant cell arteritis present after their sixth decade of life, and the peak incidence occurs in patients aged 60-80 years. However, studies show that polymyalgia rheumatica, usually in association with giant cell arteritis, can occur in younger patients, as early as the fifth decade of life, but this is rare. Therefore, consider an alternative diagnosis in patients younger than 50 years.

Clinical

History

The following are criteria for giant cell arteritis (GCA) issued by the American College of Rheumatology in 1990 (the presence of ≥3 yields a diagnostic sensitivity of 93.5% and specificity of 91.2%):10

  • Age 50 years or older
  • Newly onset localized headache
  • Temporal artery tenderness or decreased temporal artery pulse
  • ESR of at least 50 mm/h
  • Abnormal artery biopsy specimen characterized by mononuclear infiltration or granulomatous inflammation

Giant cell arteritis is a strikingly heterogeneous systemic inflammatory disorder. Presentation varies from fever of unknown origin to visual loss and limb claudication. Arterial lesions may be widespread; therefore, the varied expression of giant cell arteritis can be analyzed according to the anatomical pattern of the arteries affected. Polymyalgia rheumatica and giant cell arteritis may represent two parts of a single disease spectrum, with giant cell arteritis at the more severe end. They share certain constitutional symptoms, eg, fatigue, weight loss, and fever. Approximately 50% of patients with giant cell arteritis have features of polymyalgia rheumatica, ie, proximal stiffness, soreness, and pain. Earlier descriptions of giant cell arteritis emphasize manifestations that were attributable to involvement of the ophthalmic artery and branches of the external carotid system, but arterial lesions may be widespread.

  • Constitutional symptoms
    • Patients with giant cell arteritis or polymyalgia rheumatica frequently report malaise and fatigue. They are usually mildly febrile. The fever associated with giant cell arteritis may reach 102°F in some cases. Night sweats may occur. Thus, the presentation of fever of unknown origin is much more common with giant cell arteritis than polymyalgia rheumatica.
    • Anorexia and weight loss may be prominent features, suggesting a malignancy, in which case an age-appropriate malignancy evaluation is recommended.
  • Proximal myalgias and stiffness
    • Polymyalgia rheumatica is characterized by symmetric proximal joint and muscle aching, soreness, and stiffness. These symptoms are most prominent in the shoulder, neck, and pelvic girdles and may involve the distal joints and muscle groups. The onset of symptoms may be abrupt or insidious over weeks to months.
    • Aching and stiffness are worse in the morning and with exertion, and they may be severe and incapacitating. Muscles may be tender, disuse may lead to atrophy, and contractures may develop. Muscle strength (usually normal) is often difficult to evaluate because of pain.
    • Polymyalgia rheumatica often coexists with giant cell arteritis. In some series, 10-15% of patients with pure polymyalgia rheumatica had associated giant cell arteritis based on findings from biopsy of the temporal artery. Conversely, 50-70% of patients with giant cell arteritis had associated polymyalgia rheumatica.
    • Even when treating patients for isolated polymyalgia rheumatica, instruct the patient to report the development of headache or visual symptoms that could be caused by occult giant cell arteritis.
  • Joint symptoms
    • Most patients have poorly localized tenderness over the joints, especially the shoulders and hips.
    • Synovitis was once excluded as a feature of giant cell arteritis, but moderate bland effusions can develop in the knees and occasionally other joints (eg, shoulders, wrists).
    • Carpal tunnel syndrome and peripheral synovitis may be present in patients with polymyalgia rheumatica, potentially causing diagnostic confusion.
  • Symptoms related to vasculitis involving branches of the external carotid artery
    • Headache and scalp pain are probably the most common symptoms of giant cell arteritis, occurring in 50-75% of patients. Headache is often the first manifestation and is described as extracranial, dull, boring, and burning. Persistent and prominent temporal headaches represent a typical symptom. Patients with occipital artery involvement may have occipital pain and difficulty combing their hair or discomfort from the pressure of a pillow on the head.
    • Involvement of the posterior auricular artery may manifest as pain in the ear canal, pinna, or parotid gland.
    • Jaw claudication and pain (predominantly in the masseter muscles with chewing) are highly specific symptoms of giant cell arteritis and occur in nearly 50% of patients. Patients with involvement of the maxillary or lingual arteries may have jaw or tongue pain when chewing or talking, and tongue gangrene has been reported.
  • Symptoms related to vasculitic involvement of the ophthalmic artery and its branches
    • In patients with giant cell arteritis, decreased vision secondary to arteritis is the most common serious consequence. This occurs in 20-50% of patients who present to ophthalmologists and is the presenting symptom at diagnosis in 60% of patients with giant cell arteritis who develop visual loss.
    • A careful history from most patients who present with sudden visual loss reveals that headache, constitutional symptoms, and polymyalgia rheumatica (usually specific enough to suggest diagnosis) precede blindness in approximately 40% of patients. Even the evolution of the visual loss is often staggered, with a partial field defect progressing to complete blindness over days. If giant cell arteritis remains untreated, the second eye may become affected within 1-2 weeks.
    • Ocular manifestations vary according to the pattern of arterial branch involvement.
    • The posterior ciliary arteries are the arteries most frequently involved in giant cell arteritis; thus, ischemic optic neuritis is the most common lesion.
    • The central retinal artery supplies the retina, which is the terminal branch of the ophthalmic artery. Occlusion of the central retinal artery or its branches occurs in fewer than 10% of patients with eye involvement; therefore, retinal changes (eg, exudates, hemorrhages, vasculitis) are uncommon.
    • Amaurosis fugax occurs in approximately 10% of patients with giant cell arteritis, and, if not treated, 80% of those patients develop permanent visual loss.
    • The posterior ciliary arteries that supply the optic nerve and the muscular branches that supply the extraocular muscles also derive from the ophthalmic artery. Thus, diplopia or ptosis, observed in 5% of patients with giant cell arteritis, may precede visual loss.
    • Note that the visual abnormality that occurs can be a composite of many ischemic events occurring together in the optic nerve, extraocular muscles, chiasm, and the brain itself.
  • Symptoms related to large artery involvement
    • These symptoms are related to involvement of the aortic arch and thoracic aorta. Limited pathologic studies show giant cell arteritis in vessels with bruits; however, the frequency of aortic and aortic root involvement in giant cell arteritis, based on clinical evidence, varies between 9% and 18%.11
    • Certain clinical characteristics distinguish large vessel from cranial giant cell arteritis. Approximately 88% of large vessel involvement occurs in women. Patients typically have a younger age at onset, fewer constitutional symptoms, and a longer interval until diagnosis. Thoracic aneurysms with giant cells in the tissue can develop as late as 15 years after the diagnosis and successful treatment of giant cell arteritis. These patients are less likely to have positive temporal artery biopsy findings, headache, jaw claudication, or visual changes and are more likely to have arm claudication at disease onset.
    • The major symptoms are arm or leg claudication. Occasionally, symptoms related to intermittent or persistent brain ischemia develop and are related to a subclavian steal syndrome or narrowing of other aortic arch vessels or, rarely, even intracerebral vascular disease.
    • Abdominal aortic aneurysms can occur. Giant cell arteritis can manifest as symptoms of aortic aneurysms and intestinal infarction. For unknown reasons, renal involvement is rare.

Physical

In polymyalgia rheumatica, a striking paucity of findings is encountered during the physical examination relative to the severity of the symptoms. The same may be true for giant cell arteritis, although ocular and funduscopic evidence of ischemic disease is present in symptomatic patients.

  • Patients may present with fever.
  • Muscles may be tender.
  • Joints are tender, especially over the shoulder and pelvic girdles. Mild peripheral synovitis may be present in patients with polymyalgia rheumatica.
  • Temporal arteries are prominent, beaded, tender, and pulseless; however, a totally normal appearance of these vessels in no way excludes a diagnosis of giant cell arteritis.
  • Funduscopic examination findings are typically normal, although optic atrophy or ischemic optic neuropathy may be observed in patients with symptoms of visual loss.
  • Bruits may be heard over the carotid, axillary, or brachial arteries.

Causes

The cause of polymyalgia rheumatica and giant cell arteritis is unknown. Environmental and genetic factors likely play a significant role.

  • Genetic factors
    • Polymyalgia rheumatica and giant cell arteritis may aggregate in families.12
    • Polymyalgia rheumatica and giant cell arteritis are more common among individuals of European descent and less common among African Americans.
    • In polymyalgia rheumatica and giant cell arteritis, the frequency of the human leukocyte antigen DR4 is approximately twice that of normal controls in some series and may be an important susceptibility factor.
    • An infectious etiology has been speculated to trigger giant cell arteritis.13

More on Giant Cell Arteritis

Overview: Giant Cell Arteritis
Differential Diagnoses & Workup: Giant Cell Arteritis
Treatment & Medication: Giant Cell Arteritis
Follow-up: Giant Cell Arteritis
References
[ CLOSE WINDOW ]

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Further Reading

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Keywords

giant cell arteritis, temporal arteritis, GCA, cranial arteritis, polymyalgia rheumatica, PMR, occult giant cell arteritis, aching syndrome, Horton disease, Horton's disease, intimal hyperplasia, vasculopathy, ischemic optic retinopathy, stenotic lesions, ischemic optic retinopathy, vision loss, visual defects, fever of unknown origin, systemic vasculitis, granulomatous arteritis, limb claudication

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Contributor Information and Disclosures

Author

Mythili Seetharaman, MD, Consulting Staff, Einstein Arthritis Center, Albert Einstein Medical Center, St Christopher's Hospital for Children
Mythili Seetharaman, MD is a member of the following medical societies: American College of Rheumatology and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Stephen A Paget, MD, Chair, Division of Rheumatology, Chief, Department of Medicine, Hospital for Special Surgery; Professor, Department of Internal Medicine, Division of Rheumatology, Weill-Cornell University
Stephen A Paget, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Evan Leibowitz, MD, Fellow, Department of Internal Medicine, Division of Rheumatology, Valley Hospital
Evan Leibowitz, MD is a member of the following medical societies: Alpha Omega Alpha and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

John Varga, MD, Professor, Department of Internal Medicine, Division of Rheumatology, Northwestern University
John Varga, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Central Society for Clinical Research, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center
Lawrence H Brent, MD is a member of the following medical societies: American Association of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; Merck, Amgen, Biogen, Zimmer, Wyeth, Johnson&Johnson, Stryker, Medtronic, Zimmer.Abbott,  Ownership interest Other; West Penn Allegheny Health System Consulting fee Consulting; Alpharma Honoraria Consulting; Proctor&Gamble Grant/research funds Independent contractor

 
 
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