eMedicine Specialties > Dermatology > Diseases of the Vessels

Temporal (Giant Cell) Arteritis

Author: John G Albertini, MD, Consulting Staff, Dermatologic Surgery, The Skin Surgery Center
Coauthor(s): Victor J Marks, MD, Associate, Department of Dermatology, Section Chief, Dermatologic Surgery, Geisinger Health System; Hyland Cronin, MD, Resident, Dermatology Department, Geisinger Health System, Danville, Pennsylvania
Contributor Information and Disclosures

Updated: May 29, 2009

Introduction

Background

Temporal arteritis (TA), also known as cranial arteritis or giant cell arteritis, was first clinically recognized in 1890 when Hutchinson described an 80-year-old man whose painful inflamed temporal arteries precluded his wearing a hat. In 1932, Horton et al correlated the histopathologic features with the clinical features and applied the name arteritis temporalis. Other names include arteritis cranialis, Horton disease, granulomatous arteritis, and arteritis of the aged.

Since Horton's first description of temporal arteritis in the United States, this form of systemic vasculitis has become more widely recognized, as has the potential for serious sequelae, such as blindness and death. In 1960, Paulley and Hughes described the broad clinical spectrum and variable manifestations of the disease. Temporal arteritis is the most common form of systemic vasculitis in adults. Temporal arteritis primarily affects medium-sized arteries of the head and the neck. Most signs and symptoms reflect this distribution and include visual disturbances, headache, jaw claudication, neck pain, and scalp tenderness.

Although varied histopathologic findings have been noted over the years, temporal artery biopsy (TABx) remains the criterion standard for diagnosis of this granulomatous vasculitis. Until discovery of the anti-inflammatory properties of corticosteroids, patients with temporal arteritis received only supportive symptomatic treatment. Since 1950, steroids have revolutionized the treatment of the disease. Treatment with systemic corticosteroids produces a predictable clinical response and may prevent permanent visual loss, the most feared ischemic complication of the vasculitis.

Pathophysiology

The exact etiology of temporal arteritis remains unknown, although it is T-cell dependent and antigen driven. Temporal arteritis is classified as a systemic vasculitis, although it primarily affects the extracranial large and medium muscular arteries of the head and the neck. Both renal arteritis and aortic arteritis are rare. The vasculitis primarily damages the media and destroys the internal elastic layer. A panarteritis develops, and intimal proliferation causes luminal occlusion. The granulomatous infiltrate is largely mononuclear and consists primarily of polyclonal CD4+ T cells and macrophages. Of these lesions, 50% contain giant cells clustered near the disrupted internal elastic lamina.

Studies by O'Brien and Regan1 have focused on the pathophysiologic role of actinic damage in persons with temporal arteritis. Chronic solar damage to elastic fibers may initiate an elastase-mediated degradation of the internal elastic lamina in temporal arteries, which may evoke an autoimmune response, resulting in granulomatous vasculitis.

The model of pathogenesis developed by Weyand and Goronzy2 proposes that an as yet undetermined antigen is first encountered in the adventitia. T cells localized to this layer are activated and produce interferon gamma, which stimulates macrophage differentiation and migration. Giant cells are formed and secrete growth factors and metalloproteinases that ultimately degrade the internal elastic lamina and produce occlusive luminal hyperplasia. Potential antigens include elastin, viral or bacterial epitopes, and other extracellular matrix proteins.

Finally, numerous studies now suggest that temporal arteritis 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.3

Frequency

United States

Temporal arteritis is one of the most prevalent of the systemic vasculitis syndromes, with an estimated prevalence of 200 cases per 100,000 people older than 50 years and an incidence of 20-30 new cases per 100,000 persons older than 50 years. Although the condition had been considered less common among Hispanic persons, recent evidence has challenged this notion.

International

The incidence, age, and sex distribution in northern Europe is similar to that in the United States, likely because of immigration patterns. Latitude is an important risk factor, with a prevalence in Sweden twice that of Spain or Italy.

Mortality/Morbidity

Mortality from temporal arteritis is extremely uncommon, especially with timely diagnosis and treatment. In 1972, Wilkinson and Russell4 reviewed 12 autopsy cases and reported a high prevalence of severe involvement of the superficial temporal, vertebral, ophthalmic, and posterior ciliary arteries and less frequent severe disease of the internal carotid, external carotid, and central retinal arteries. Intracranial arteries were never involved.

These patients, who died during the active phase of temporal arteritis, demonstrated a high prevalence of monocular blindness, occipital blindness, and brainstem strokes. Insignificant vasculitis was rarely identified in aortic, suprarenal, and coronary arteries and did not contribute to death. A close correlation existed between the amount of elastic tissue in susceptible arteries and the severity of the vasculitis. Subsequent morbidity and mortality from temporal arteritis reflect this anatomical distribution of vessels.

An insidious onset of the disease usually progresses to include fatigue, malaise, fever, weight loss, polymyalgia rheumatica, and flulike symptoms. Headache, scalp tenderness, focal arterial tenderness, jaw claudication, and visual loss may develop, and these findings indicate vascular involvement.

Race

Temporal arteritis primarily affects whites, specifically those of northern European descent. Scandinavians have the highest prevalence, and temporal arteritis is rare in African Americans. The gene for HLA-DRB1-04 has been identified as a risk factor for temporal arteritis. Although the condition had been considered less common among Hispanic persons, recent evidence has challenged this notion.

Sex

Females are affected in 80% of cases. Smoking increases the temporal arteritis risk 6-fold in women, while diabetes reduces the risk by half.

Age

Temporal arteritis is extremely uncommon in younger age groups. The average age at onset is in the sixth to seventh decade of life, with a mean age at onset of 69 years. It affects the same older population as the commonly associated polymyalgia rheumatica.

Clinical

History

  • Temporal arteritis often manifests in an insidious manner with vague constitutional symptoms such as malaise, weight loss, fever, and fatigue.
  • The classic manifestations are fever, anemia, headache, and an elevated erythrocyte sedimentation rate (ESR).
  • Polymyalgia rheumatica (PMR) may develop either before or after the arteritis.
  • Disease onset may also be abrupt. The diagnosis should be strongly suspected in whites older than 50 years of northern European descent, especially women, who report temporal headache, tender scalp, jaw claudication, and/or visual changes.
  • Visual disturbances include diplopia, blurred vision, amaurosis fugax, and blindness in either one eye or both eyes.
  • Symptoms signaling peripheral neuropathy or aortic involvement are rare.
  • A history of chronic sun exposure may be relevant.
  • In 2003, Amor-Dorado et al5 reported a previously unrecognized high incidence of audiovestibular disturbances such as vestibular dysfunction and/or hearing impairment in their temporal arteritis patients. Another rare complication involved lower extremity involvement with rapidly progressive claudication signaling compromised vascular flow. Surgery was often required.

Physical

  • The examination should include an assessment of vital signs and blood pressure in both arms to rule out aortic arch involvement.
    • The scalp may be focally or generally tender. Temporal arteries may be tender, inflamed, dilated, thickened, or cordlike.
    • The arteries may be pulsatile, particularly early in the disease course.
    • Bruits may signal partial occlusion.
    • In severe cases, scalp skin or oral mucosal ulceration develops.
  • Neurologic examination may reveal a neuropathy.
  • Ophthalmologic examination reveals ischemic optic neuropathy in early stages of the disease and optic atrophy later in the disease course. Immediate referral is critical to ensure prompt diagnosis of reversible retinal ischemia from ophthalmic or posterior ciliary artery involvement.
  • Audiovestibular examination may be considered.

Causes

The exact etiology of temporal arteritis remains unknown. See Pathophysiology.

More on Temporal (Giant Cell) Arteritis

Overview: Temporal (Giant Cell) Arteritis
Differential Diagnoses & Workup: Temporal (Giant Cell) Arteritis
Treatment & Medication: Temporal (Giant Cell) Arteritis
Follow-up: Temporal (Giant Cell) Arteritis
References
[ CLOSE WINDOW ]

References

  1. O'Brien JP, Regan W. Actinically degenerate elastic tissue is the likely antigenic basis of actinic granuloma of the skin and of temporal arteritis. J Am Acad Dermatol. Feb 1999;40(2 Pt 1):214-22. [Medline].

  2. Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. Jul 10 2003;349(2):160-9. [Medline].

  3. Salvarani C, Pipitone N, Boiardi L, Hunder GG. Do we need treatment with tumour necrosis factor blockers for giant cell arteritis?. Ann Rheum Dis. May 2008;67(5):577-9. [Medline].

  4. Wilkinson IM, Russell RW. Arteries of the head and neck in giant cell arteritis. A pathological study to show the pattern of arterial involvement. Arch Neurol. Nov 1972;27(5):378-91. [Medline].

  5. Amor-Dorado JC, Llorca J, Garcia-Porrua C, Costa C, Perez-Fernandez N, Gonzalez-Gay MA. Audiovestibular manifestations in giant cell arteritis: a prospective study. Medicine (Baltimore). Jan 2003;82(1):13-26. [Medline].

  6. Ellen H, Nusser J. Which clinical features and lab findings increase the likelihood of temporal arteritis?. The Journal of Family Practice. 2008;57:119-120.

  7. Schmidt WA, Kraft HE, Vorpahl K, Völker L, Gromnica-Ihle EJ. Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med. Nov 6 1997;337(19):1336-42. [Medline].

  8. Salvarani C, Silingardi M, Ghirarduzzi A, et al. Is duplex ultrasonography useful for the diagnosis of giant-cell arteritis?. Ann Intern Med. Aug 20 2002;137(4):232-8. [Medline].

  9. Alberts M, Mosen D. Diagnosing temporal arteritis: duplex vs biopsy. Quarterly Journal of Medicine. 2007;100:785-789.

  10. Bley TA, Reinhard M, Hauenstein C, Markl M, Warnatz K, Hetzel A. Comparison of duplex sonography and high-resolution magnetic resonance imaging in the diagnosis of giant cell (temporal) arteritis. Arthritis Rheum. Aug 2008;58(8):2574-8. [Medline].

  11. Marano SR, Fischer DW, Gaines C, Sonntag VK. Anatomical study of the superficial temporal artery. Neurosurgery. Jun 1985;16(6):786-90. [Medline].

  12. Wolff K et al. Fig 243-4. In: Fitzpatrick's Dermatology In General Medicine. 7th. 2008:2291.

  13. DAnesh-Meyer H, Eagle R, Kubis K, Savineo P, and Sergott R. Low Diagnositc Yield with Second Biopsies in Suspected Giant Cell Arteritis. Journal of Neuro-Opthalmology. 2000;20:213-215.

  14. Levin L, Nuiederkohr R. A Bayesian Analysis of the True Sensitivity of a Temporal Artery Biopsy. Investigative Opthalmology & Visual Science. 2007;48:675-680.

  15. Armstrong AT, Tyler WB, Wood GC, Harrington TM. Clinical importance of the presence of giant cells in temporal arteritis. J Clin Pathol. May 2008;61(5):669-71. [Medline].

  16. Martinez-Taboada VM, Rodriguez-Valverde V, Carreno L, Lopez-Longo J, Figueroa M, Belzunegui J. A double-blind placebo controlled trial of etanercept in patients with giant cell arteritis and corticosteroid side effects. Ann Rheum Dis. May 2008;67(5):625-30. [Medline].

  17. Cuizhen K, Scula C. Mesenteric Involvement in Giant Cell Arteritis. An Underrecognized Complication?: Analysis of a Case Series with Clinicoanatomic Correlation. Medicine. 2008;87:45-51.

  18. Albert DM, Searl SS, Craft JL. Histologic and ultrastructural characteristics of temporal arteritis. The value of the temporal artery biopsy. Ophthalmology. Oct 1982;89(10):1111-26. [Medline].

  19. Albertini JG, Ramsey ML, Marks VJ. Temporal artery biopsy in a dermatologic surgery practice. Dermatol Surg. Jun 1999;25(6):501-8. [Medline].

  20. Bevan AT, Dunnill MS, Harrison MJ. Clinical and biopsy findings in temporal arteritis. Ann Rheum Dis. May 1968;27(3):271-7. [Medline].

  21. Birkhead NC, Wagener HP, Shick RM. Treatment of temporal arteritis with adrenal corticosteroids; results in fifty-five cases in which lesion was proved at biopsy. J Am Med Assoc. Mar 9 1957;163(10):821-7. [Medline].

  22. Brennan J, McCrary JA 3rd. Diagnosis of superficial temporal arteritis. Ann Ophthalmol. Aug 1975;7(8):1125-9. [Medline].

  23. Chmelewski WL, McKnight KM, Agudelo CA, Wise CM. Presenting features and outcomes in patients undergoing temporal artery biopsy. A review of 98 patients. Arch Intern Med. Aug 1992;152(8):1690-5. [Medline].

  24. Fauchald P, Rygvold O, Oystese B. Temporal arteritis and polymyalgia rheumatica. Clinical and biopsy findings. Ann Intern Med. Dec 1972;77(6):845-52. [Medline].

  25. Fernandez-Herlihy L. Temporal arteritis: clinical aids to diagnosis. J Rheumatol. Dec 1988;15(12):1797-801. [Medline].

  26. Hall S, Persellin S, Lie JT, O'Brien PC, Kurland LT, Hunder GG. The therapeutic impact of temporal artery biopsy. Lancet. Nov 26 1983;2(8361):1217-20. [Medline].

  27. Hedges TR 3rd, Gieger GL, Albert DM. The clinical value of negative temporal artery biopsy specimens. Arch Ophthalmol. Aug 1983;101(8):1251-4. [Medline].

  28. Huston KA, Hunder GG, Lie JT, Kennedy RH, Elveback LR. Temporal arteritis: a 25-year epidemiologic, clinical, and pathologic study. Ann Intern Med. Feb 1978;88(2):162-7. [Medline].

  29. Kelley JS. Doppler ultrasound flow detector used in temporal artery biopsy. Arch Ophthalmol. May 1978;96(5):845-6. [Medline].

  30. Kent RB 3rd, Thomas L. Temporal artery biopsy. Am Surg. Jan 1990;56(1):16-21. [Medline].

  31. Klein RG, Campbell RJ, Hunder GG, Carney JA. Skip lesions in temporal arteritis. Mayo Clin Proc. Aug 1976;51(8):504-10. [Medline].

  32. Lam BL, Wirthlin RS, Gonzalez A, Dubovy SR, Feuer WJ. Giant cell arteritis among Hispanic Americans. Am J Ophthalmol. Jan 2007;143(1):161-3. [Medline].

  33. Levine SM, Hellmann DB. Giant cell arteritis. Curr Opin Rheumatol. Jan 2002;14(1):3-10. [Medline].

  34. Nordborg E, Nordborg C. Giant cell arteritis: epidemiological clues to its pathogenesis and an update on its treatment. Rheumatology (Oxford). Mar 2003;42(3):413-21. [Medline].

  35. Nordborg E, Nordborg C. Giant cell arteritis: strategies in diagnosis and treatment. Curr Opin Rheumatol. Jan 2004;16(1):25-30. [Medline].

  36. Schumacher HR, Klippel JH, Koopman WJ, eds. Vasculitis. In: Primer on the Rheumatic Diseases. 10th ed. Atlanta, Ga: Arthritis Foundation; 1993:136-48.

  37. Seko Y. Giant cell and Takayasu arteritis. Curr Opin Rheumatol. Jan 2007;19(1):39-43. [Medline].

  38. Sorensen S, Lorenzen I. Giant-cell arteritis, temporal arteritis and polymyalgia rheumatica. A retrospective study of 63 patients. Acta Med Scand. 1977;201(3):207-13. [Medline].

  39. Weyand CM, Bartley GB. Giant cell arteritis: new concepts in pathogenesis and implications for management. Am J Ophthalmol. Mar 1997;123(3):392-5. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

temporal arteritis, giant cell arteritis, arteritis temporalis, arteritis cranialis, Horton disease, Horton's disease, granulomatous arteritis, arteritis of the aged, cranial arteritis, systemic vasculitis, TA, giant cell arteritis, temporal arteritis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

John G Albertini, MD, Consulting Staff, Dermatologic Surgery, The Skin Surgery Center
John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology and American College of Mohs Micrographic Surgery and Cutaneous Oncology
Disclosure: Nothing to disclose.

Coauthor(s)

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.

Hyland Cronin, MD, Resident, Dermatology Department, Geisinger Health System, Danville, Pennsylvania
Disclosure: Nothing to disclose.

Medical Editor

Russell Hall, MD, Chief, Professor, Department of Internal Medicine, Division of Dermatology, Duke University
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: Genetech Grant/research funds Principle Investigator; Centecor  Grant/research funds Principle Investigator

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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.

Managing Editor

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: Johnson and Johnson stock holder dividends; Amgen stock holder dividends; Forest Lab, Inc stock holder dividends; Galaxo Smith Klein stock holder dividends; Covidien stock holder dividends; Novartis Grant/research funds Consulting; Biolex  sub-investigator

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

RELATED EMEDICINE ARTICLES
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.