eMedicine Specialties > Radiology > Vascular/Interventional
Temporal Arteritis
Updated: Oct 2, 2008
Introduction
Background
Temporal arteritis, or giant cell arteritis, is a common systemic vasculitis of unknown etiology. In 1890, Hutchinson originally described the condition as inflamed and swollen temporal arteries. In 1932, Horton expanded the definition. In general, temporal arteritis can be thought of as a vasculitis involving medium-to-large arteries originating from the aorta. Although it was originally believed to be a rare entity, it is more commonly recognized today.1,2,3,4,5
Pathophysiology
The exact cause of temporal arteritis (giant cell arteritis) is not well delineated. It is characterized by a granulomatous inflammatory process that is most pronounced along the internal elastic lamina of arterial walls. Temporal artery biopsy demonstrates a predominance of mononuclear cells or multinucleated giant cells with fragmentation of the intima. Inflammation may be followed by intimal proliferation and eventual stenosis or occlusion of the involved arterial segment.
The clinical findings are a result of decreased blood flow and include jaw or tongue claudication; extremity stiffness; scalp tenderness; and visual changes with anterior ischemic optic neuropathy (AION), amaurosis, or optic atrophy leading to blindness in as many as 60% of patients.
Frequency
United States
Temporal arteritis (giant cell arteritis) occurs in an estimated 15-30 per 100,000 persons. It almost exclusively affects individuals older than 50 years and is believed to have a prevalence as high as 1 case per 500 individuals in this age group.
Mortality/Morbidity
To the author's knowledge, no controlled studies have been performed to compare patients with untreated temporal arteritis (giant cell arteritis) and patients with treated arteritis. However, definitive evidence suggests that mortality rates in treated patients and those in untreated patients do not differ.
Race
The incidence of temporal arteritis (giant cell arteritis) is lower in blacks than in whites.
Sex
The female-to-male ratio for temporal arteritis (giant cell arteritis) is 4-6:1.
Age
Although temporal arteritis (giant cell arteritis) occurs almost exclusively in patients older than 50 years, well-documented cases have been reported in patients as young as 40 years.
Anatomy
Temporal arteritis (giant cell arteritis) can affect any medium or large artery, but the clinical signs and symptoms are usually related to the inflammation that occurs in the branches of the external carotid artery.6 Temporal arteritis is caused by inflammation of the internal elastic lamina of the arterial wall. Intradural cranial arteries have no elastic lamina. For this reason, temporal arteritis seldom affects the cerebral circulation.
In the region of the parotid gland, the external carotid artery terminates by dividing into the superficial temporal and maxillary arteries. The superficial temporal artery crosses the zygomatic process of the temporal bone and divides into frontal and parietal branches that traverse the scalp. The superficial temporal artery typically does not develop atherosclerotic plaques, and the vessel is reportedly observed on 88% of cerebral angiograms.7 In addition, angiography can demonstrate areas of constriction, beading, and microaneurysm formation that are fairly specific for temporal arteritis. The most common sites for abnormalities to occur anatomically and on imaging studies are in the distal subclavian, proximal axillary, brachial, brachiocephalic, and femoral arteries.
Presentation
Multiple symptoms may be present in the patient with temporal arteritis and are usually attributable to decreased blood flow in the affected anatomic area. These symptoms include headache, which may be unilateral; scalp tenderness; jaw and/or tongue claudication; and changes in vision, including blindness. Constitutional symptoms may be present as well, and these are likely the result of the inflammatory nature of the disease. Fever, anorexia, and stiffness of the neck, trunk, and extremities may be present.
In 1990, the American College of Rheumatology established criteria for the classification of temporal arteritis. In one study, investigators compared 214 patients with temporal arteritis with 593 control subjects and determined that if patients had 3 of the 5 criteria, temporal arteritis could be diagnosed with a sensitivity of 93.5% and a specificity of 91.2%. The 5 criteria are as follows: (1) age older than 50 years, (2) new onset of localized headache, (3) temporal artery tenderness or decreased temporal arterial pulse, (4) increased erythrocyte sedimentation rate (>50 mm/h), and (5) arterial biopsy showing necrotizing arteritis characterized by a predominance of mononuclear cell infiltrates or a granulomatous process.8,9
Preferred Examination
Currently, temporal artery biopsy is the criterion standard for the diagnosis of temporal arteritis (giant cell arteritis). A negative biopsy finding does not exclude the diagnosis. Angiography can be used when biopsy results are negative, or it can be used to help guide biopsy by demonstrating areas of abnormality.10,11 When performed, angiography is typically directed at the large branch vessels of the proximal aorta and extracranial carotid branch vessels. The temporal arteries are depicted well in almost 90% of patients. In patients with proximal artery stenoses, angioplasty can be used in addition to corticosteroid therapy for symptomatic relief.12
Although angiography is one of the best-studied techniques, it is invasive and inconvenient. As a result, less-invasive procedures for evaluating the arterial anatomy have been sought. Magnetic resonance angiography (MRA) has results comparable to those of angiography in evaluating medium-to-large vessels. In some reported cases, MRA has successfully depicted disease in the temporal arteries. As the sensitivity of MRA continues to improve, it will likely become a more realistic method for evaluating stenotic lesions attributed to temporal arteritis.13,14,15
Studies have revealed the benefit of ultrasonography in the diagnosis of temporal arteritis.16,17,18,19 Characteristic changes, including stenoses and occlusions of temporal artery segments and a dark halo around the vessel, have been reliably observed in patients with temporal arteritis. Doppler flow studies have also been performed, with promising results.20
A study of positron emission tomography (PET) scanning evaluated18 F-glucose uptake and demonstrated a sensitivity of 56%, a specificity of 98%, and a positive predictive value of 93% for the diagnosis of giant cell arteritis or polymyalgia rheumatica.21
Limitations of Techniques
Angiography is an excellent study; however, it is limited for temporal arteritis by the invasive nature of the examination and by the risks associated with the administration of contrast material.
Magnetic resonance angiography is a noninvasive examination, but it has limited use in evaluating smaller vessels, and imaging artifacts may result in false-positive results. In addition, larger vessels with mildly thickened walls can be missed.
Ultrasonography may not depict minor vascular changes or diseased vessels, such as intrathoracic vessels, that are not amenable to ultrasonography.
Differential Diagnoses
Arteritis, Giant Cell
Arteritis, Takayasu
Fibrous Dysplasia
Other Problems to Be Considered
Atherosclerotic disease affects the temporal arteries in rare cases, and its changes may resemble those associated with temporal arteritis.
Radiography
Findings
Radiographs are of no use in diagnosing temporal arteritis.
Computed Tomography
Findings
Thickening of the arterial walls, stenosis, or occlusion may be demonstrated on contrast-enhanced CT scans.
Degree of Confidence
The findings can be observed in a variety of other disease processes.
False Positives/Negatives
CT commonly fails to depict mild inflammatory changes in the vessels. CT is not useful for the evaluation of small-vessel disease. In older persons, disease processes such as atherosclerotic disease are far more common than temporal arteritis and may result in similar CT findings.
Magnetic Resonance Imaging
Findings
MRI findings for temporal arteritis (giant cell arteritis) include loss of the normal flow void in affected vessels from occlusion or slow flow associated with disease. Enhancement of the arterial wall may be observed after the administration of gadolinium-based contrast material.22,23,24 Magnetic resonance angiography (MRA) may also demonstrate stenoses, irregularity of the vessel wall, and beading or thickening of the vessel wall.
Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.
NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.
Degree of Confidence
The findings can be observed in a variety of other disease processes.
False Positives/Negatives
MRI commonly will miss mild inflammatory changes of vessels. MRI is not useful for the evaluation of small-vessel disease. In the elderly, disease processes such as atherosclerotic disease are far more common than temporal arteritis and may result in similar MRI findings.
Ultrasonography
Findings
Ultrasonography can be used to evaluate small vessels such as the temporal arteries. Findings include stenoses and occlusion of the vessels. A characteristic hypoechoic halo has been described as surrounding the affected vessel that disappears after corticosteroid therapy. Ultrasonography is also useful in guiding biopsy.
Degree of Confidence
These findings can be observed with a variety of other disease processes. Findings may be negative in patients with minimal involvement of the temporal arteries. Ultrasonography cannot be used to evaluate vessels such as intrathoracic arteries that are more amenable to angiography or MRI.
False Positives/Negatives
Though unusual, atherosclerotic disease involving the temporal arteries may have an appearance similar to that of temporal arteritis. Minimally involved vessels may appear normal.
Nuclear Imaging
Findings
Positron emission tomography (PET) scanning has been used to evaluate unusual involvement that cannot be evaluated by means of surgical biopsy or ultrasonography.
Degree of Confidence
PET cannot be used to distinguish between the increased uptake observed with temporal arteritis and that observed in polymyalgia rheumatica.
Angiography
Findings
Angiography is an invasive test with inherent risks associated with the procedure and with the administration of contrast material. Findings consist of the involvement of small-to-moderate vessels. Angiography can demonstrate areas of constriction, beading, and microaneurysm formation that are fairly specific for temporal arteritis (giant cell arteritis). The occlusion of vessels and stenoses that are amenable to treatment may also be observed.
The most common sites for abnormalities to occur anatomically and on imaging studies are in the distal subclavian, proximal axillary, brachial, brachiocephalic, and femoral arteries. Atherosclerotic disease is a common finding in the older population; however, narrowings observed with atherosclerotic disease are typically short, segmental, and irregular, whereas stenoses in temporal arteritis are smooth, long, segmental, and tapered.
Degree of Confidence
Similar findings may be observed in patients with Takayasu arteritis and in those with atherosclerotic disease. Temporal artery biopsy is more definitive than angiography, and it can be guided by the arteriographic findings.
False Positives/Negatives
False-negative results may occur in a few patients in whom the temporal arteries are not well visualized.
Intervention
Angioplasty and stent placement have been used in patients with ischemic symptoms who were not adequately treated with corticosteroid therapy. (As many as 58% of patients receiving long-term steroid therapy will have a major adverse effect. These include avascular necrosis of the hip, diabetes, congestive heart failure, peptic ulcers, hypertension, fractures, and depressive psychosis.)25
Medicolegal Pitfalls
- The failure to consider the diagnosis of temporal arteritis (giant cell arteritis) in an older patient with systemic complaints is the most likely medical pitfall.
- The failure to diagnose temporal arteritis with the prompt initiation of corticosteroid therapy may lead to ischemia and blindness.
- Ruling out temporal arteritis on the basis of a negative radiologic imaging study alone is a pitfall.
Special Concerns
- No radiologic finding is specific for the diagnosis of temporal arteritis (giant cell arteritis) alone. Imaging studies are helpful in determining the extent of involvement and in identifying unsuspected areas of involvement.
- Special care must be taken when invasive procedures such as arteriography are performed, because complications may occur.
- Patients with temporal arteritis are older than other patients, and they may have concomitant illnesses that increase the risk of a procedure.
- Patients with atherosclerotic disease or renal insufficiency have an increased risk of procedural complications.
- The risk of a potentially serious reaction to the contrast material also must be taken into consideration.
References
Danesh-Meyer HV, Savino PJ. Giant cell arteritis. Curr Opin Ophthalmol. Nov 2007;18(6):443-9. [Medline].
Schmidt WA. Takayasu and temporal arteritis. Front Neurol Neurosci. 2006;21:96-104. [Medline].
Tatò F, Hoffmann U. Giant cell arteritis: a systemic vascular disease. Vasc Med. 2008;13(2):127-40. [Medline].
Tehrani R, Ostrowski RA, Hariman R, Jay WM. Giant cell arteritis. Semin Ophthalmol. Mar-Apr 2008;23(2):99-110. [Medline].
Cantini F, Niccoli L, Nannini C, Bertoni M, Salvarani C. Diagnosis and treatment of giant cell arteritis. Drugs Aging. 2008;25(4):281-97. [Medline].
Thielen KR, Wijdicks EF, Nichols DA. Giant cell (temporal) arteritis: involvement of the vertebral and internal carotid arteries. Mayo Clin Proc. May 1998;73(5):444-6. [Medline].
Elliott PD, Baker HL Jr, Brown AL Jr. The superficial temporal artery angiogram. Radiology. Mar 1972;102(3):635-8. [Medline].
Hunder GG, Arend WP, Bloch DA. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum. Aug 1990;33(8):1065-7. [Medline].
Matteson EL, Gold KN, Bloch DA. Long-term survival of patients with giant cell arteritis in the American College of Rheumatology giant cell arteritis classification criteria cohort. Am J Med. Feb 1996;100(2):193-6. [Medline].
Devauchelle-Pensec V, Jousse S, Destombe C, Saraux A. Epidemiology, imaging, and treatment of giant cell arteritis. Joint Bone Spine. May 2008;75(3):267-72. [Medline].
Melson MR, Weyand CM, Newman NJ, Biousse V. The diagnosis of giant cell arteritis. Rev Neurol Dis. Summer 2007;4(3):128-42. [Medline].
Dellaripa PF, Eisenhauer AC. Bilateral percutaneous balloon angioplasty of the axillary arteries in a patient with giant cell arteritis and upper extremity ischemic symptoms not responsive to corticosteroids. J Rheumatol. Jul 1998;25(7):1429-33. [Medline].
Botella-Estrada R, Sammartin O, Martinez V. Magnetic resonance angiography in the diagnosis of a case of giant cell arteritis manifesting as scalp necrosis. Arch Dermatol. Jul 1999;135(7):769-71. [Medline].
Harada S, Mitsunobu F, Kodama F. Giant cell arteritis associated with rheumatoid arthritis monitored by magnetic resonance angiography. Intern Med. Aug 1999;38(8):675-8. [Medline].
Mitomo T, Funyu T, Takahashi Y. Giant cell arteritis and magnetic resonance angiography. Arthritis Rheum. Sep 1998;41(9):1702. [Medline].
Schmidt WA, Kraft HE, Vorpahl K. Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med. Nov 6 1997;337(19):1336-42. [Medline].
Myers KA, Farquhar DR. Ultrasonography in temporal arteritis. N Engl J Med. Mar 12 1998;338(11):760; discussion 761. [Medline].
Schmidt WA, Kraft HE, Borkowski A. Color duplex ultrasonography in large-vessel giant cell arteritis. Scand J Rheumatol. 1999;28(6):374-6. [Medline].
Hunder GG, Weyand CM. Sonography in giant-cell arteritis. N Engl J Med. Nov 6 1997;337(19):1385-6. [Medline].
Puechal X, Chauveau M, Menkes CJ. Temporal Doppler-flow studies for suspected giant-cell arteritis. Lancet. Jun 3 1995;345(8962):1437-8. [Medline].
Blockmans D, Stroobants S, Maes A. Positron emission tomography in giant cell arteritis and polymyalgia rheumatica: evidence for inflammation of the aortic arch. Am J Med. Feb 15 2000;108(3):246-9. [Medline].
Anders HJ, Sigl T, Sander A. Gadolinium contrast magnetic resonance imaging of the temporal artery in giant cell arteritis. J Rheumatol. Oct 1999;26(10):2287-8. [Medline].
Bley TA, Uhl M, Carew J, Markl M, Schmidt D, Peter HH, et al. Diagnostic value of high-resolution MR imaging in giant cell arteritis. AJNR Am J Neuroradiol. Oct 2007;28(9):1722-7. [Medline].
Khoury JA, Hoxworth JM, Mazlumzadeh M, Wellik KE, Wingerchuk DM, Demaerschalk BM. The Clinical Utility of High Resolution Magnetic Resonance Imaging in the Diagnosis of Giant Cell Arteritis: A Critically Appraised Topic. Neurologist. Sep 2008;14(5):330-335. [Medline].
Nesher G, Sonnenblick M, Friedlander Y. Analysis of steroid related complications and mortality in temporal arteritis: a 15-year survey of 43 patients. J Rheumatol. Jul 1994;21(7):1283-6. [Medline].
Keywords
temporal arteritis, Horton giant cell arteritis, Horton disease, Horton's disease, Horton's giant cell arteritis, giant cell aortic arteritis, giant cell aortitis, juvenile temporal arteritis, central nervous system vasculitis, CNS vasculitis, giant cell arteritis, systemic vasculitis, temporal arteries, GCA, cranial arteritis, granulomatous arteritis
Contributor Information and Disclosures
Author
Anthony W Allen, MD, Chief, Interventional Radiology, Brooke Army Medical Center; Associate Professor of Radiology, Uniformed Services University of the Health Sciences
Anthony W Allen, MD is a member of the following medical societies: American College of Radiology
Disclosure: Nothing to disclose.
Coauthor(s)
Timothy Biega, MD, Staff Physician, Department of Radiology, Tripler Regional Medical Center
Disclosure: Nothing to disclose.
Manish K Varma, MD, Chief of Interventional Radiology, Department of Radiology, Tripler Army Medical Center
Manish K Varma, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, and Radiological Society of North America
Disclosure: Nothing to disclose.
Medical Editor
Anthony Watkinson, MD, Professor of Interventional Radiology, The Peninsula Medical School; Consultant and Senior Lecturer, Department of Radiology, The Royal Devon and Exeter Hospital, UK
Anthony Watkinson, MD is a member of the following medical societies: Radiological Society of North America, Royal College of Radiologists, and Royal College of Surgeons of England
Disclosure: Nothing to disclose.
Pharmacy Editor
Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
Managing Editor
Douglas M Coldwell, MD, PhD,, Principal, Coldwell Associates. Interventional Radiologist, Jane Phillips Medical Center, Bartlesville, OK
Douglas M Coldwell, MD, PhD, is a member of the following medical societies: American Association for Cancer Research, American College of Radiology, American Heart Association, American Physical Society, American Roentgen Ray Society, Society of Cardiovascular and Interventional Radiology, Southwest Oncology Group, and Special Operations Medical Association
Disclosure: Sirtex, Inc. Consulting fee Speaking and teaching
CME Editor
Robert M Krasny, MD, Consulting Staff, Department of Radiology, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.
Chief Editor
Kyung J Cho, MD, FACR, William Martel Professor of Radiology, Interventional Radiology Fellowship Director, University of Michigan Health System
Kyung J Cho, MD, FACR is a member of the following medical societies: American College of Radiology, American Heart Association, American Medical Association, American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.
Related eMedicine topics
Temporal (Giant Cell) Arteritis (Dermatology)
Temporal Arteritis (Emergency Medicine)
Temporal/Giant Cell Arteritis (Neurology)
Giant Cell Arteritis (Ophthalmology)
Arteritis, Giant Cell (Radiology)
Giant Cell Arteritis (Rheumatology)
Clinical guidelines
Diagnosis and treatment of headache .
Institute for Clinical Systems Improvement. 1998 Aug (revised 2007 Jan). 72 pages. NGC:005845
Treatment of primary headache: chronic daily headache. Standards of care for headache diagnosis and treatment .
National Headache Foundation. 2004. 8 pages. NGC:004143
Headache.
American College of Radiology. 1996 (revised 2006). 8 pages. NGC:005120
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