eMedicine Specialties > Ophthalmology > Hematologic & Cardiovascular Disorders

Giant Cell Arteritis

Author: Manolette R Roque, MD, MBA, DPBO, FPAO, President and CEO, Chief of Service, Ocular Immunology and Uveitis, Consulting Staff, Cornea and Refractive Surgery, Eye Republic Ophthalmology Clinic; General Manager, Ophthalmic Consultants Philippines Co; Consulting Staff, CME Liaison, Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center
Coauthor(s): Barbara L Roque, MD, Full Partner, Ophthalmic Consultants Philippines Co, Chief of Service, Pediatric Ophthalmology and Strabismus, Consulting Staff, Orbit and Eye Plastics, Eye Republic Ophthalmology Clinic; Elisabetta Miserocchi, MD, Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Harvard Medical School; C Stephen Foster, MD, FACS, FACR, FAAO, 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
Contributor Information and Disclosures

Updated: Oct 23, 2008

Introduction

Background

Giant cell arteritis (GCA) is also called temporal arteritis, cranial arteritis, and granulomatous arteritis. GCA is a systemic inflammatory vasculitis of unknown etiology that affects medium- and large-sized arteries.1,2,3,4,5,6,7,8,9,10,11,12,13,14 It is a disease of elderly persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications. Visual loss is one of the most significant causes of morbidity in GCA. Permanent visual impairment may occur in as many as 60% of patients. Newly recognized GCA should be considered a true neuro-ophthalmic emergency. Prompt treatment with steroids can prevent blindness and other vascular sequelae of GCA.15

Relationship with polymyalgia rheumatica

A close relationship exists between GCA and polymyalgia rheumatica (PMR).16,17,18,19,20,21,22,23 Despite numerous reports associating these 2 disease entities, the precise nature for this association is poorly understood and currently unknown. However, it is evident that both conditions affect similar patient populations and frequently affect the same individual. Several authors have suggested that these 2 diseases are actually different stages of a single disease spectrum. See Special Concerns.

Criteria for diagnosis

The American College of Rheumatology criteria (1990) for the diagnosis of GCA are as follows2,24 :

  • Age at onset of 50 years
  • New headache
  • Abnormalities of the temporal arteries
  • Erythrocyte sedimentation rate (ESR) of 50 mm/h
  • Positive results of a temporal artery biopsy (vasculitis characterized by a predominance of mononuclear infiltrates or granulomas, usually with multinucleated giant cells)

Pathophysiology

GCA typically involves inflammation of the aortic arch and its branches, but almost any artery of the body as well as some veins may be affected occasionally. The inflammation tends to involve the arteries in a segmental or patchy manner, although long portions of arteries may be involved.25,26,27 The likely determinant of arterial susceptibility to GCA is the presence and/or quantity of internal elastic lamina within the vessel wall. For example, intracranial cerebral vasculature is not affected in GCA because these vessels lack an internal elastic lamina.

The extracranial vertebral arteries, superficial temporal arteries, posterior ciliary arteries, and ophthalmic arteries are the most commonly involved arteries. The internal carotid, external carotid, and central retinal arteries are affected somewhat less frequently. In some postmortem studies, lesions were found in the proximal and distal aorta, subclavian, brachial, and abdominal arteries. Intracranial arteries are involved infrequently.

Histopathology (see Histologic Findings) in GCA reveals inflammatory infiltrate surrounding a fragmented internal elastic lamina within the media of an arterial wall. The infiltrate consists predominantly of mononuclear cells with giant cell formation. The mechanism is believed to be related to dysfunction of cellular immunity, but the etiology is unknown (see Causes).

Frequency

United States

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

In Olmstead County, Minnesota, 125 cases were identified over a 42-year review 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 has been noted.

International

The annual incidence in northern European countries has been reported to be more than 20 cases per 100,000 people. 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.28

Ostberg reported that autopsy studies performed on 889 postmortem cases revealed arteritis (eg, temporal artery, aorta) in 1.6%, suggesting that GCA may be more common than is clinically apparent29

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 period30

Mortality/Morbidity

In the milder form of GCA, patients may complain only of generalized muscle aches and pains and unusual fatigue.1,2 These may be mistaken for symptoms of polymyalgia rheumatica. Intermittent claudication occurs in about 50% of patients. During chewing of firm foods such as meat, fatigue or discomfort of the jaw muscles is noted. In a small percentage of patients, claudication of the tongue or throat develops with eating and repeated swallowing. 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.

  • Visual symptoms are present in about 33% of patients; 40-50% are transient (amaurosis fugax and diplopia) and 50-60% are permanent. Regarded as one of the more serious complications of GCA is the onset of blindness from involvement of the ophthalmic artery. Permanent visual loss may be partial or complete and may occur without warning; about 50% are unilateral and 50% are bilateral. Varied visual symptoms including blurring of vision, diplopia, and loss of vision occur in 36-60% of patients.
  • Rarely, 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).
  • Aortic aneurysms, aortic regurgitation, and aortic dissection occur less commonly. Evans and colleagues reported aortic aneurysms occurring in 15% of patients at a median of 6 years after the GCA initially was diagnosed. Two thirds were thoracic aortic aneurysms, with the majority located in the ascending aorta. Almost 33% developed symptomatic aortic regurgitation.
  • Involvement of major vessels (aorta) predisposes patients to higher risks for death. In Evans and colleagues' report, 50% of those with thoracic aortic aneurysms died suddenly from aortic dissection.

Race

Incidence rates appear to be higher in Caucasians of European descent. This condition is less common in African Americans and Asians.31

Sex

Women are 2-4 times more likely to have GCA than men.

Age

Age is the most important risk factor for GCA.

  • GCA occurs mostly in patients older than 50 years, with incidence increasing with age and peaking in the eighth decade. The disease is rare in patients younger than 50 years.
  • Although the increasing incidence of GCA after age 50 years implies a relationship to aging, the meaning of this observation is not fully understood.

Clinical

History

The onset of GCA may be either abrupt or insidious. Usually, the symptoms have been present for weeks or months before the diagnosis is established. Constitutional symptoms, including anorexia, fatigue, and weight loss, are present in most patients and may be an initial finding. It is important to be aware of characteristic ophthalmic and systemic histories to effectively diagnose GCA. 

See related CME at Diagnosis and Management in Neuro-Ophthalmology. 

  • Ophthalmic history
    • The most dramatic symptom of GCA, visual loss, results directly from the inflammation of the medium- and large-sized cranial vessels32,33,34,35,36,37,38,39,40,41,42 Around 50% of patients with GCA eventually experience visual symptoms (eg, transient visual blurring, diplopia, eye pain, sudden vision loss). Transient repeated episodes of blurred vision are usually reversible, but sudden loss of vision is an ominous sign and is almost always permanent. Vision loss incidence, either partial or complete, is variably reported to be 10-60%.
    • The most common cause of vision loss is anterior ischemic optic neuropathy (AION). This results from ischemia of the optic nerve head, supplied mainly by the posterior ciliary arteries. Most AION is nonarteritic (87-91%) in nature. A history of sudden painless loss of vision frequently accompanies the patient with AION.
  • Systemic history
    • GCA may begin with symptoms of anorexia, fever, malaise, myalgia, night sweat, and weight loss. These prodromal symptoms may occur for a few days and may even stretch out to weeks.1,43,44,45
    • The hallmark symptom of GCA is its new-onset localized headache. It usually is localized to the temporal or occipital area. This headache occasionally may be diffuse or bilateral.

Physical

  • Ophthalmic manifestations
    • The most common cause of vision loss is AION.46,47,48,49,50,51,52,53,54,55 Examination of the fundus may reveal optic disc edema, with or without splinter hemorrhages along the disc margin. Arteritic AION, as in GCA, typically presents with a chalky white edematous optic disc. Automated visual field testing typically reveals an inferior altitudinal defect, inferior nasal sectorial defect, or central scotoma.
    • Other important vascular ophthalmic presentations of GCA56,57,58,59 include posterior ischemic (retrobulbar) optic neuropathy, central retinal artery occlusion,60 branch retinal artery occlusion, and choroidal ischemia.61,62,63 Neuro-ophthalmic manifestations64 of GCA include diplopia, ptosis,65 nystagmus, internuclear ophthalmoplegia (INO),66 and pupillary abnormalities.67
  • Systemic manifestations
    • The hallmark symptom of GCA is new-onset localized headache, which usually is localized to the temporal or occipital area. This headache occasionally may be diffuse or bilateral. Of interest is the scalp tenderness that accompanies this headache, especially over the temporal region. The tenderness often can be induced by only very gentle pressure. Hypersensitivity or hyperesthesia (unusual discomfort from a very mild stimulus) like gently stroking the patient's hair results in a characteristic complaint of pain commonly seen with migraine, whereas with GCA a more painful or tender sensation can be elicited by gentle pressure. Rizzo anecdotally describes this as the single most important clinical finding for GCA.11 Other cranial symptoms include temporal tenderness or pulselessness, jaw claudication, facial pain, earache, toothache, tongue and palate pain, and odynophagia. Dudenhoefer described scalp necrosis with GCA seen in 2 elderly patients.68,69,70
    • Other clinical features of GCA manifest as pulselessness and/or tenderness and inflammation along the course of the temporal artery and bruits in the cranial or neck area. In addition, typical symptoms include jaw claudication, and atrophy of temporal and tongue muscles. Temporal artery blood flow measurements may be reduced.
    • Cerebrovascular disease occurs in 1-25% of patients and is believed to be the most common cause of death in patients with GCA. Neurologic problems associated with GCA include myopathy, neuro-otologic syndromes, neuropsychiatric syndromes, peripheral neuropathies, and seizures. Cardiovascular, pulmonary, gastrointestinal, renal, and dermatologic manifestations also may occur.71,72,73,74,75

Causes

A variety of causes (ie, genetic, infectious, autoimmune) have been suggested for GCA, but the etiology remains unknown.76,77,27,78

  • Genetic: Several reports of familial aggregation, association with the HLA-DR4 haplotype, and an apparent increased frequency of these conditions in northern Europe and in persons in the United States with similar ethnic backgrounds suggest a genetic or hereditary predisposition.
  • Infectious: Some epidemiological observations, reports, and studies using DNA detection techniques implicate Chlamydia pneumoniae, Mycoplasma pneumoniae, and parvovirus B19 as the impetus for the destructive inflammation.79
  • Autoimmune: 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.80,54 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.

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
Multimedia: Giant Cell Arteritis
References
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Further Reading

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Keywords

giant cell arteritis, GCA, temporal arteritis, cranial arteritis, Horton's disease, Horton disease, Horton arteritis, Horton's arteritis, granulomatous arteritis, polymyalgia rheumatica, vasculitis, migraine headache

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Contributor Information and Disclosures

Author

Manolette R Roque, MD, MBA, DPBO, FPAO, President and CEO, Chief of Service, Ocular Immunology and Uveitis, Consulting Staff, Cornea and Refractive Surgery, Eye Republic Ophthalmology Clinic; General Manager, Ophthalmic Consultants Philippines Co; Consulting Staff, CME Liaison, Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center
Manolette R Roque, MD, MBA, DPBO, FPAO is a member of the following medical societies: American Academy of Ophthalmic Executives, American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, American Society of Ophthalmic Administrators, American Uveitis Society, International Ocular Inflammation Society, Philippine Medical Association, Philippine Ocular Inflammation Society, and Philippine Society of Cataract and Refractive Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Barbara L Roque, MD, Full Partner, Ophthalmic Consultants Philippines Co, Chief of Service, Pediatric Ophthalmology and Strabismus, Consulting Staff, Orbit and Eye Plastics, Eye Republic Ophthalmology Clinic
Disclosure: Nothing to disclose.

Elisabetta Miserocchi, MD, Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Harvard Medical School
Disclosure: Nothing to disclose.

C Stephen Foster, MD, FACS, FACR, FAAO, 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 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, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

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

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
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 Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

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.

 
 
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