eMedicine Specialties > Radiology > Vascular/Interventional

Arteritis, Giant Cell

Author: Guruswami Arunagiri, MD, FRCS, Consulting Staff, Department of Ophthalmology, Geisinger Medical Center
Coauthor(s): S Santhi, MD, Staff Physician, Department of Internal Medicine, Geisinger Medical Center; VP Sukumar, MBBS, MD, FRCR, Specialist Registrar, Department of Radiology, Oxford Radcliffe Hospitals NHS Trust, UK
Contributor Information and Disclosures

Updated: Apr 23, 2008

Introduction

Background

Giant cell arteritis is a disease of elderly persons, the incidence of which increases with increasing age. The mean age of onset is 70 years. The inflammation is a necrotizing obliterative vasculitis of large and medium-sized vessels.¹

For excellent patient education resources, visit eMedicine's Circulatory Problems Center. Also, see eMedicine's patient education article Phlebitis.

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Pathophysiology

Giant cell arteritis is a systemic disease mainly involving the arteries that originate from the arch of aorta. However, any vessel in the body can be affected. The superficial temporal, vertebral, ophthalmic, and posterior ciliary arteries are more commonly affected than the internal and external carotid arteries. Intracranial arteries other than those involving the orbits are less commonly affected. Cases involving the proximal distal aorta and the subclavian and abdominal arteries have been reported. The inflammation is segmental, and therefore, skip lesions are seen in giant cell arteritis.²

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Temporal/Giant Cell Arteritis (Neurology)

Giant Cell Arteritis (Ophthalmology)

Frequency

United States

In Olmsted County, Minnesota, the prevalence is 17 cases per 100,000 population aged 50 years or older. In Tennessee, the prevalence is 1.58 cases per 100,000 population aged 50 years or older.

International

In Scandinavia, the prevalence is 17 cases per 100,000 population aged 50 years or older.

Mortality/Morbidity

Giant cell arteritis is an obliterative vasculitis that can result in blindness, stroke, or death.

Race

Giant cell arteritis is reported more commonly in whites than in those of other races. This difference may be due to bias in access to medical care. However, giant cell arteritis affects persons of all races.

Sex

Giant cell arteritis is 2-3 times more common in women than in men.

Age

The mean age of onset is 70 years.

Anatomy

Giant cell arteritis affects large and medium-sized arteries with elastic lamina. Any blood vessel in the body can be affected. However, it is more common in the blood vessels that originate from the arch of the aorta. The superficial temporal, vertebral, ophthalmic, and posterior ciliary arteries are commonly involved.

Presentation

Clinical features of giant cell arteritis consist of both systemic and neuro-ophthalmic findings.

Systemic features include the following: (1) new-onset temporal headache, (2) scalp tenderness, (3) jaw claudication, (4) anorexia, (5) weight loss, (6) low-grade fever, (7) depression, and (8) polymyalgia rheumatica.

Neuro-ophthalmic features include the following: (1) anterior ischemic optic neuropathy; (2) posterior ischemic optic neuropathy; (3) transient blurring of vision; (4) palsies of the third, fourth, and sixth cranial nerves; (5) retinal arterial occlusion; and (6) orbital pseudotumor.

Giant cell arteritis causes rapid visual loss. Blindness may occur in hours. Loss of vision is mostly due to posterior ciliary artery occlusion and not central retinal artery occlusion.

Neurologic manifestations include cerebrovascular accidents (CVAs).

Skin manifestations include scalp necrosis.

Oral-cavity manifestations include tongue claudication and tongue necrosis.^3,4

Related eMedicine topics:

Polymyalgia Rheumatica

Sudden Visual Loss

Giant Cell Arteritis (Ophthalmology)

Preferred Examination

Color Doppler ultrasonography is a method for assessing blood flow qualitatively and quantitatively. In the presence of arteritis, the sonograms show a hypoechoic halo due to edema of the arterial wall.⁵ The sensitivity and specificity of this finding are not well established. The advantages of this technique are that it is simple, it is noninvasive, and it can be used to examine several vessels, superficial and deep. Another advantage of this technique is that follow-up scans can be obtained to assess the response to steroids. The hypoechoic shadow becomes mid echoic in about 2 weeks. With fibrosis, the shadow becomes hyperechoic.^{6,7,8,9,10,11,12}

Other nonradiologic investigations include a determination of the blood sedimentation rate and temporal artery biopsy (see Images below and Images 1-3 in Multimedia). The criterion standard is the temporal artery biopsy (see Limitations of Techniques below).

Limitations of Techniques

The criterion used to diagnose giant cell arteritis with color Doppler ultrasonography is the presence of a halo sign. This is not seen in all patients, and it may also be seen in healthy persons. The halo has also been reported in polyarteritis nodosa. Therefore, the criterion standard is the temporal artery biopsy.

Differential Diagnoses

Abdominal Aortic Aneurysm, Rupture

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