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Giant Cell Arteritis (Temporal Arteritis)

  • Author: Mythili Seetharaman, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Nov 04, 2015
 

Background

Giant cell arteritis (GCA), or temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that occurs in older persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications. GCA is the most common form of systemic vasculitis in adults. Other names for GCA include arteritis cranialis, Horton disease, granulomatous arteritis, and arteritis of the aged.

GCA typically affects the superficial temporal arteries—hence the term temporal arteritis. In addition, GCA most commonly affects the ophthalmic, occipital, vertebral, posterior ciliary, and proximal vertebral arteries. It has also been shown to involve medium- and large-sized vessels, including the aorta and the carotid, subclavian, and iliac arteries.[1, 2, 3]

Histopathologically, GCA is marked by transmural inflammation of the intima, media, and adventitia of affected arteries, 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. (See Pathophysiology.)

Age and female sex are established risk factors for GCA, a genetic component seems likely, and infection may have a role (see Etiology). One school of thought considers GCA and polymyalgia rheumatica to be different manifestations of the same disease process, while others see them as closely related but different diseases.[4]

Common signs and symptoms of GCA reflect the involvement of the temporal artery and other medium-sized arteries of the head and the neck and include visual disturbances, headache, jaw claudication, neck pain, and scalp tenderness. Constitutional manifestations, such as fatigue, malaise, and fever, may also be present. (See Presentation.)

GCA should always be considered in the differential diagnosis of a new-onset headache in patients 50 years of age or older with an elevated erythrocyte sedimentation rate. Temporal artery biopsy remains the criterion standard for diagnosis of this granulomatous vasculitis (see the image below).

Hematoxylin- and eosin-stained superficial tempora Hematoxylin- and eosin-stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of GCA shown here include intimal thickening with luminal stenosis, mononuclear inflammatory cell infiltrate with media invasion and necrosis, and giant cell formation in the media.

Visual loss is one of the most significant causes of morbidity in GCA. Permanent visual impairment may occur in as many as 20% of patients, and, in some cases, GCA can cause bilateral blindness.[5] Newly recognized GCA should be considered a true neuro-ophthalmic emergency.

Prompt initiation of treatment may prevent blindness and other potentially irreversible ischemic sequelae of GCA.[6] Corticosteroids are the mainstay of therapy. In steroid-resistant cases, drugs such as cyclosporine, azathioprine, or methotrexate may be used as steroid-sparing agents. The typical patient with GCA remains on steroid therapy for roughly 2 years. (See Treatment and Medication.)

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Pathophysiology

Giant cell arteritis (GCA) is primarily a disease of cell-mediated immunity, which is thought to arise as a maladaptive response to endothelial injury. Actinic damage to the temporal artery from chronic sun exposure has been proposed as one source of the injury.[7] The adventitia is the likely site of initial immunologic injury and is considered the immunological center of the disorder, while the intima and media are the histological center.

The primary inflammatory response involves the internal elastic lamina within the media of the arterial wall. The subsequent release of cytokines within the arterial vessel wall can attract macrophages and multinucleated giant cells. In turn, activated CD4+ T helper cells respond to an antigen presented by macrophages, which gives diseased vessels their characteristic histology. The inflammation tends to occur in a segmental or patchy manner, although long portions of arteries may be involved.[8, 9]

Systemic manifestations are likely related to the inflammatory process and cytokine elaboration. End-organ involvement relates to hyperplasia and occlusion of the arteries serving those organs.

Concentric intimal hyperplasia is an important underlying pathologic lesion in GCA. Researchers assume that intimal hyperplasia occurs when the blood vessel wall responds to injury and that it is a repair mechanism.

Platelet-derived growth factor (PDGF) is important in stimulating intimal hyperplasia. In GCA, PDGF derives from macrophages and giant cells, and this distinguishes GCA from other vasculopathies. In atherosclerotic disease, for example, PDGF is produced mostly by resident smooth muscle cells rather than monocytes.

Intimal macrophages also produce vascular endothelial growth factor (VEGF), which promotes intimal proliferation. Medial macrophages generate metalloproteinases, leading to the destruction of vascular elements, including the internal elastic lamina.[10] Adventitial macrophages produce interleukin-6 (IL-6), augmenting the inflammatory response. This results in inflammation with local vascular damage and intimal hyperplasia, leading to stenosis and occlusion.[11]

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 demonstrated that different adhesion molecules might regulate how leukocytes and endothelial cells interact in different temporal artery layers.[12]

A study by Maugeri et al showed that patients with GCA had increased expression of platelets expressing P-selectin, of platelet-Nph and platelet-Mo aggregates, and of Nph and Mo expressing tissue factor.[13] Activated platelets and white cells could cause vessel inflammation and thromboembolic events.[14]

Elastin

A cellular immune reaction to elastin has been implicated in the pathogenesis of GCA.[15] In support of the hypothesis that elastin is the inciting antigen, disease severity has been shown to correlate with the amount of elastic tissue within the vessels.[16] For example, intracranial arteries lack an internal elastic lamina, and GCA does not cause a widespread intracranial cerebral vasculitis.

This hypothesis also is supported by histopathologic findings of a disrupted, fragmented internal elastic lamina in affected vessels and the presence of characteristic giant cells, which may contain elastic fiber fragments, close to the internal elastic lamina. Along with elevated serum levels of neutrophil elastase, deposition of elastase along the damaged internal elastic lamina has been described.

Pattern of involvement

The superficial temporal artery is involved in most patients, providing a convenient biopsy site, but this is only the "tip of the iceberg." GCA commonly affects arteries in the following pattern:

  • Common, external, and internal carotid artery involvement is usually extracranial; rarely, proximal intracranial segments have been affected
  • Intraorbital branches, especially the posterior ciliary and ophthalmic arteries, are commonly affected
  • Vertebral arteries are involved as frequently as the superficial temporal arteries in fatal cases, although basilar artery involvement is rare
  • Vertebral arteritis is extracranial, but it may extend intracranially for roughly 5 mm beyond dural penetration
  • Subclavian, axillary, and proximal brachial arterial involvement produces a characteristic angiographic pattern of vasculitis, consisting of long, smooth, stenotic segments that alternate with nonstenotic segments and tapered occlusions
  • Involvement of the ascending aorta can lead to aortic rupture, and coronary arteritis may result in myocardial infarction (MI)
  • Less often, the descending aorta and mesenteric, renal, iliac, and femoral arteries can be affected, with attendant complications of intestinal infarction, renal infarction, crural infarction, and ischemic mononeuropathies [17, 18]
  • Pulmonary arterial involvement has also been described
  • Some veins may be affected occasionally

The most common cause of vision loss in GCA is anterior ischemic optic neuropathy (AION). This results from ischemia of the optic nerve head, which is supplied mainly by the posterior ciliary arteries.

Relationship with polymyalgia rheumatica

A close relationship exists between GCA and polymyalgia rheumatica.[19, 20] About half of persons with GCA have underlying polymyalgia rheumatica, and about 15% of individuals with polymyalgia rheumatica develop GCA. The precise nature of this association is poorly understood. Several authors have suggested that these 2 diseases are actually different stages

Finally, numerous studies now suggest that GCA consists of various clinical subsets rather than one uniform disease. Variable expression of different cytokine profiles likely determines the clinical manifestations. Tumor necrosis factor (TNF) and, more recently, interleukin 6, have been recognized to may play a major role in the pathophysiology of temporal arteritis.[21]

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Etiology

The exact etiology of giant cell arteritis (GCA) remains unknown. Despite increased understanding of the inflammatory cascade responsible for the disease process, the initial event that triggers the cascade remains uncertain. Genetic, environmental, and autoimmune factors have been identified.

Reports of familial aggregation,[22] association with the HLA-DR4 haplotype, and an apparent higher frequency of these conditions in northern Europe and in persons in the United States with similar ethnic backgrounds suggest a genetic or hereditary predisposition. GCA is less common among African Americans. A possible association between Toll-like receptor 4 gene polymorphism and susceptibility to biopsy-proven GCA has been found.[23]

Epidemiological observations, reports, and studies using DNA detection techniques have implicated Chlamydia pneumoniae, Mycoplasma pneumoniae, and parvovirus B19 as the impetus for the destructive inflammation.[24, 25] Nevertheless, it is generally accepted that these infectious agents are only "innocent bystanders."

The immune system (both cellular and humoral) has been implicated in the pathogenesis of GCA. The granulomatous histopathology of GCA has suggested the presence of an antigen-driven disease with local T-cell and macrophage activation in or near elastic tissue in the arterial walls with an important role of the proinflammatory cytokines.[26, 27] It may begin as a foreign body giant cell attack on calcified internal elastic membrane in the arteries and on calcified atrophic parts of the aortic media.

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Epidemiology

United States statistics

The reported incidence of GCA ranges from approximately 0.5 to 27 cases per 100,000 people aged 50 years or older.[14] The annual incidence is higher in northern areas of the United States.

A review from Olmsted County, Minnesota identified 125 cases over 42 years, representing an average annual incidence rate of 17.8 cases per 100,000 population aged 50 years and older and a prevalence of persons with active or remitted GCA of 200 cases per 100,000 population aged 50 years or older. A regular cyclical pattern in incidence over 20 years was noted.[28]

International statistics

The prevalence of GCA depends heavily on the number of individuals aged 50 years or older; the mean age of onset is 75 years. Countries with a lower life expectancy have a lower prevalence.

The annual incidence in northern European countries has been reported to be more than 20 cases per 100,000 people. A United Kingdom study reported an incidence of 22 per 100,000.[29] Scandinavian countries have reported the highest incidence.

The annual incidence in southern European countries has been reported to be less than 12 cases per 100,000 people. In Lugo, Spain, the average annual incidence for the population aged 50 years and older was 10 cases per 100,000 people.

In Saskatoon, Saskatchewan, Canada and the surrounding area, the estimated incidence of GCA for the population aged older than 50 years was 9.4 cases per 100,000 people.[30] A series comprising all adult subjects undergoing autopsy at two hospitals in southern Sweden revealed arteritis in 1.6% of 889 cases, suggesting that GCA may be more common than is clinically apparent.[31]

The incidence of GCA in Saudi Arabia is probably less than in the United States and Western Europe. In 1998, Bosley and Riley reported only 4 positive biopsy results from 72 temporal artery biopsies performed over a 15-year period in Saudi patients.[32]

Racial differences in incidence

Although existing epidemiologic studies are limited because they have been performed on predominantly white populations, the results of these studies suggest that GCA primarily affects whites, specifically those of northern European descent. Scandinavians have the highest prevalence. GCA is less common in African Americans and Asians.[33] Although GCA had been considered less common among Hispanic persons,[34] recent evidence has challenged this notion.

Sexual and age-related differences in incidence

The female-to-male ratio in GCA is roughly 3.7:1. Smoking increases the risk 6-fold in women, while diabetes reduces the risk by half.

Age is the most important risk factor for GCA. The disease is rare in patients younger than 50 years. In those 50 years and older, the incidence increases with age, peaking in the eighth decade. The age range in one series of 166 cases proven by temporal artery biopsy was 55-92 years. The median age of onset is 75 years. GCA is the most common systemic vasculitis affecting elderly patients.

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Prognosis

With prompt, adequate therapy, full recovery is the rule. Symptoms from temporal arteritis improve within days of treatment. Corticosteroids can usually be tapered within the first 4-6 weeks and eventually discontinued. The reduced rate of neuro-ophthalmologic complications in recent years reflects improved recognition and treatment; blindness is now a rare complication.

Although the overall course of the disease is one of progressive improvement and eventual complete resolution, the clinical course is highly variable. The average duration of treatment is 2 years; however, some patients require treatment for 5 years or more.[15, 11] Morbidity from steroid therapy is often worse than the underlying disease. Rarely, patients do not respond to steroid therapy or doses cannot be tapered. Cytotoxic or immunosuppressive drugs have been recommended in such cases, but more data are needed.

In a study that included 35 patients with both polymyalgia rheumatic and temporal arteritis, Narváez and colleagues found that no clinical feature was associated with increased risk of relapse. However, they reported that multivariate analysis identified the following as significant risk factors associated with long duration of steroid therapy[35] :

  • Older age at diagnosis
  • Female sex
  • Higher baseline erythrocyte sedimentation rate
  • Lower daily corticosteroid dose

The prognosis for patients with untreated GCA is extremely poor. These patients may suffer blindness, or death from myocardial infarction, stroke, or dissecting aortic aneurysm. Complications Ophthalmic complications are common and include visual disturbances, amaurosis fugax, diplopia, and blindness from involvement of the ophthalmic artery. Visual loss may be temporary or permanent and partial or complete in either one eye or both eyes. Blindness usually occurs abruptly and painlessly. Ischemic optic neuritis with eventual optic atrophy is the most common cause of visual loss and occurs in 15% of patients.

Vision damage that occurs before initiation of therapy is often irreversible,[36] especially in patients who have other ischemic complications.[37] In patients whose vision loss is initially unilateral, the second eye may become affected even after the initiation of treatment (possibly because those vessels have already been affected by arteritis) in 6-13% of patients.[38]

Progression of vision loss despite the initiation of high-dose corticosteroid therapy typically occurs within the first 5-6 days of treatment if therapy is going to fail.[39, 40] Risk factors for progressive visual loss despite steroid therapy include older age, elevated CRP level, and optic disc swelling.[41]

Nervous system alterations are found in as many as 30% of patients; 14% have either mononeuritis or polyneuropathy, and 7% have transient ischemic attacks or strokes.[42] Strokes from GCA may lead to multi-infarct dementia. Solans-Laqué et al report 7 patients with biopsy-proven giant cell arteritis who presented with stroke or multi-infarct dementia.[43] Rare but serious complications include myocardial infarction (MI) and visceral organ ischemia (eg, small-bowel infarction).[44, 45, 17]

Complications of long-term steroid treatment that may occur in patients with GCA include the following:

  • Vertebral body compression fracture (26%)
  • Symptomatic steroid myopathy (11%)
  • Steroid psychosis (3%)

Mortality/morbidity

An observational cohort study using a United Kingdom primary care database found that GCA is associated with increased risks for MI, stroke, and peripheral vascular disease. The hazard ratios were 2.06 for MI, 1.28 for stroke, and 2.13 for peripheral vascular disease. Hazard ratios were more pronounced in the first month after the diagnosis of GCA.[46]

GCA leads to death by stroke or MI in roughly 2% of cases. As would be expected, the topographic extent and severity of the vasculitis is greater in fatal than in nonfatal cases. More difficult to quantify are the number of patients whose deaths are related directly or indirectly to chronic corticosteroid use and its attendant complications (eg, hip fracture).

A population-based cohort study from South Australia found that mortality rates in patients with biopsy-proven GCA were similar to those in the general population. Cardiovascular disease was the most common cause of death, followed by infection and cancer. An increased risk of death from infection was more common in the first year, which may be related to immunosuppression from higher doses of corticosteroids at that stage of the disease.[47]

The inflammatory process may weaken the aortic wall, leading to localized aneurysm formation, aortic annular dilatation, and aortic regurgitation. Narrowing or occlusion of the branch vessels of the thoracic aorta (clinically referred to as aortic arch syndrome) may be found in 9-14% of cases, producing symptoms similar to those of Takayasu arteritis (decreased upper extremity pulses and blood pressure, arm or leg claudication, Raynaud phenomenon, transient ischemic attacks, coronary ischemia, and abdominal angina).

Evans and colleagues reported aortic aneurysms occurring in 15% of patients at a median of 6 years after the initial diagnosis of GCA. Two thirds were thoracic aortic aneurysms, with the majority located in the ascending aorta. Almost 33% of patients developed symptomatic aortic regurgitation. Approximately 50% of patients with thoracic aortic aneurysms died suddenly from aortic dissection.

In very rare cases, GCA involves the central nervous system (CNS), producing abnormal mental status, seizures, or strokes. This is despite the fact that GCA for the most part affects only vessels with an elastica, which intradural blood vessels do not have. However, involvement of the aortic arch vessels, including the subclavian arteries, can lead to subclavian steal syndrome and brain ischemia.

Peripheral nerve involvement is rare in GCA. For unknown reasons, renal involvement is also rare.

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Patient Education

Education is the most important step in helping the patient appreciate the clinical facets of this illness, the potential adverse effects of the therapy, and the need for monitoring. Patients who experienced visual loss prior to the initiation of therapy should be cautioned that despite therapy, the lost vision may not be regained.

Patients must be instructed about the risk of complications and symptomatic relapses. Advise patients to immediately consult a physician if they experience symptoms of transient blurring of vision because of the possibility of impending attacks of GCA or transient ischemic attack.

Patients must understand the importance of strictly adhering to their steroid dose schedule and the possible need for ancillary interventions, such as dietary restrictions, to reduce the incidence of steroid-related adverse effects. To avoid misunderstandings, inform patients and their families about vertebral compression fracture and other potential complications of steroid therapy that can occur even with proper therapy.

Patients should be informed that they carry a lifelong risk for the development of large vessel disease, particularly aortic aneurysms. The need for long-term followup should be stressed.

For patient education information, see the Eye and Vision Center and Headache and Migraine Center.

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Contributor Information and Disclosures
Author

Mythili Seetharaman, MD Consultant Rheumatologist, OAA; Clinical Assistant Professor, Thomas Jefferson University Hospital, St Christopher's Hospital for Children

Mythili Seetharaman, MD is a member of the following medical societies: American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, Sigma Xi

Disclosure: Nothing to disclose.

John G Albertini, MD Private Practice, The Skin Surgery Center; Clinical Associate Professor (Volunteer), Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine; President-Elect, American College of Mohs Surgery

John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery

Disclosure: Received grant/research funds from Genentech for investigator.

Stephen A Paget, MD Physician-in-Chief Emeritus, Joseph P Routh Professor of Medicine, New York Hospital, Weill Cornell Medical College; Program Director, Cornell Arthritis and Multipurpose Arthritis and Musculoskeletal Diseases Center (MAMDC), Hospital for Special Surgery

Stephen A Paget, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Manolette R Roque, MD, MBA, FPAO Section Chief, Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief, Ocular Immunology and Uveitis, International Eye Institute, St Luke's Medical Center Global City; Senior Eye Surgeon, The LASIK Surgery Clinic; Director, AMC Eye Center, Alabang Medical Center

Manolette R Roque, MD, MBA, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Philippine Medical Association, American Uveitis Society, International Ocular Inflammation Society, Philippine Ocular Inflammation Society, American Society of Ophthalmic Administrators, American Academy of Ophthalmic Executives, Philippine Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Acknowledgements

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of 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 for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Richard J Caselli, MD Professor, Department of Neurology, Mayo Medical School; Chair, Department of Neurology, Mayo Clinic of Scottsdale

Disclosure: Nothing to disclose.

Steve Charles, MD Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Hyland Cronin, MD Resident Physician, Dermatology Department, Geisinger Health System

Disclosure: Nothing to disclose.

Ann G Egland, MD Consulting Staff, Department of Operational and Emergency Medicine, Walter Reed Army Medical Center

Disclosure: Nothing to disclose.

Dirk M Elston, MD Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Gino A Farina, MD, FACEP, FAAEM Associate Professor of Emergency Medicine, Hofstra North Shore LIJ School of Medicine and 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.

Kilbourn Gordon III, MD, FACEP Urgent Care Physician

Kilbourn Gordon III, MD, FACEP is a member of the following medical societies: American Academy of Ophthalmology and Wilderness Medical Society

Disclosure: Nothing to disclose.

Russell Hall, MD J Lamar Callaway Professor And Chair, Department of Dermatology, Duke University Medical Center, Duke University School of Medicine

Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, American Society for Clinical Investigation, and Society for Investigative Dermatology

Disclosure: Novan Consulting fee Consulting; Stieffel, a GSK company Consulting fee Consulting; Society for Investigative Dermatology Salary Board membership

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.

Leslie W Jackson, MD, LTC, MC Assistant Professor, Department of Medicine, Uniformed Services University of the Health Sciences; Assistant Chief, Rheumatology Service, Department of Medicine, Walter Reed Army Medical Center

Disclosure: Nothing to disclose.

B Mark Keegan, MD, FRCPC Associate Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee Consulting; Bionest Consulting fee Consulting; Bristol Meyers Squibb Consulting fee Consulting; Caridian BCT Grant/research funds Other

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.

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.

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.

Victor J Marks, MD Associate, Department of Dermatology, Section Chief, Dermatologic Surgery, Geisinger Health System

Victor J Marks, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physicians, American Medical Association, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Jorge E Mendizabal, MD Consulting Staff, Corpus Christi Neurology

Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neurology, American Headache Society, National Stroke Association, and Stroke Council of the American Heart Association

Disclosure: Nothing to disclose.

Elisabetta Miserocchi, MD Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Harvard Medical School

Disclosure: Nothing to disclose.

Julia R Nunley, MD Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Arun Ramachandran State University of New York Upstate Medical University

Arun Ramachandran is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Tarakad S Ramachandran, MBBS, FRCP(C), FACP, FRCP Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP, FRCP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, RoyalCollegeofPhysicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Boeringer-Ingelheim Honoraria Speaking and teaching

Barbara L Roque, MD Full Partner, Ophthalmic Consultants Philippines Co; Service Chief, Pediatric Ophthalmology and Strabismus, Department of Ophthalmology, Asian Hospital and Medical Center; Active Staff, International Eye Institute, St Luke's Medical Center Global City; Visiting Ophthalmologist, AMC Eye Center, Alabang Medical Center

Barbara L Roque, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Cataract and Refractive Surgery, Philippine Academy of Ophthalmology, Philippine Society of Cataract and Refractive Surgery, and Philippine Society of Pediatric Ophthalmolo

Disclosure: Nothing to disclose.

Hampton Roy Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

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: Medscape Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed Director, Regional MS Center of Excellence, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, National Multiple Sclerosis Society, and Sigma Xi

Disclosure: Nothing to disclose.

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of Medscape Reference gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.

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Hematoxylin- and eosin-stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of GCA 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.
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 GCA resulted in ischemic necrosis of the lower limb, necessitating amputation.
Hematoxylin and eosin stain, low power. Temporal artery. Note the thrombosis in the lumen, intimal hyperplasia, and infiltration of the arterial wall muscular layers with inflammatory cells. A multinucleated giant cell is seen internal to the muscularis at the area of the internal elastic lamina (upper right).
Anterior ischemic optic neuropathy. Image courtesy of Richard Kho, MD, Q.C. Eye Center, Quezon City, Philippines.
Branch retinal vein occlusion in a patient with giant cell arteritis. Image courtesy of Manolette Roque, MD, Roque Eye Clinic, Manila, Philippines.
 
 
 
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