You are in: eMedicine Specialties > Radiology > VASCULAR/INTERVENTIONAL Arteritis, Giant CellArticle Last Updated: Apr 23, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 9
  Author: Guruswami Arunagiri, MD, FRCS, Consulting Staff, Department of Ophthalmology, Geisinger Medical Center Guruswami Arunagiri is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, National Multiple Sclerosis Society, and North American Neuro-Ophthalmology Society Coauthor(s): S Santhi, MD, Staff Physician, Department of Internal Medicine, Geisinger Medical Center; VP Sukumar, MD, Specialist Registrar, Department of Radiology, Oxford Radcliffe Hospitals NHS Trust Editors: 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; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Douglas M Coldwell, MD, PhD, Professor and Chief of Interventional Radiology, Professor of Radiology and Surgery, University of Missouri at Columbia; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Kyung J Cho, MD, FACR, William Martel Professor of Radiology, Fellowship Program Director, Department of Radiology, Division of Interventional Radiology, University of Michigan Medical School Author and Editor Disclosure Synonyms and related keywords: temporal arteritis, cranial arteritis, necrotizing obliterative vasculitis INTRODUCTIONSection 2 of 9
  BackgroundGiant 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. PathophysiologyGiant 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 FrequencyUnited StatesIn 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. InternationalIn Scandinavia, the prevalence is 17 cases per 100,000 population aged 50 years or older. Mortality/MorbidityGiant cell arteritis is an obliterative vasculitis that can result in blindness, stroke, or death. RaceGiant 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. SexGiant cell arteritis is 2-3 times more common in women than in men. AgeThe mean age of onset is 70 years. AnatomyGiant 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. Clinical DetailsClinical 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 Preferred ExaminationColor 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 1-3). The criterion standard is the temporal artery biopsy (see Limitations of Techniques below). Limitations of TechniquesThe 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. DIFFERENTIALSSection 3 of 9
Abdominal Aortic Aneurysm, Rupture CT SCANSection 4 of 9
FindingsCT is not useful in diagnosing giant cell arteritis. However, CT may be used to diagnose complications due to giant cell arteritis, such as a CVA. MRISection 5 of 9
FindingsMRI is not useful in diagnosing giant cell arteritis. However, MRI may be used to diagnose complications due to giant cell arteritis, such as a CVA. ULTRASOUNDSection 6 of 9
FindingsColor Doppler ultrasonography is a method of assessing blood flow qualitatively and quantitatively. In the presence of arteritis, the sonograms show a hypoechoic halo due to edema of the arterial wall. The sensitivity and specificity of this finding is 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. Degree of ConfidenceThe criteria used to diagnose giant cell arteritis with color Doppler ultrasonography is the presence of a halo sign. The specificity and sensitivity of this sign is not well established. This is not present 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 temporal artery biopsy. INTERVENTIONSection 7 of 9
The treatment of giant cell arteritis is steroid therapy. Most patients need to receive steroids for at least 1 year. However, steroids have several adverse effects. Hypertension, diabetes, and osteoporosis are among the main adverse effects. Patients should be carefully monitored for these sequelae.13, 14 Medical/Legal Pitfalls
MULTIMEDIASection 8 of 9
REFERENCESSection 9 of 9
Article Last Updated: Apr 23, 2008 | |||||||||||||||||||||
