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Clinical manifestations of giant cell (temporal) arteritis

William P Docken, MD
James T Rosenbaum, MD
Section Editors
Jonathan Trobe, MD
Gene G Hunder, MD
Deputy Editor
Monica Ramirez Curtis, MD, MPH


Giant cell arteritis (GCA) is categorized as a vasculitis of large- and medium-sized vessels. GCA is designated as a vasculitis of this caliber because it can involve the aorta and great vessels, and because it shares some histopathologic features with Takayasu arteritis, the other major “large vessel” vasculitis. Systemic symptoms are common in GCA and vascular involvement can be widespread. However, it is the targeting of the tiny muscular arteries from cranial branches of the aortic arch that gives rise to many of the most characteristic symptoms of GCA. The most feared complication of GCA, visual loss, is one potential consequence of such cranial arteritis.

The clinical manifestations of GCA will be reviewed here. The pathogenesis, diagnosis, and treatment of this disorder are discussed separately. (See "Pathogenesis of giant cell (temporal) arteritis" and "Diagnosis of giant cell (temporal) arteritis" and "Treatment of giant cell (temporal) arteritis".)


Giant cell arteritis (GCA) is the most common systemic vasculitis [1]. In the United States population, the lifetime risk of developing GCA has been estimated at approximately 1 percent in women and 0.5 percent in men [2].

The greatest risk factor for developing GCA is aging. The disease almost never occurs before age 50, and its incidence rises steadily thereafter, peaking between the ages of 70 to 79 [3], with over 80 percent of patients older than 70 years of age [4,5]. One study found the mean age at incidence of GCA to be 76.7 years [6].

In addition to age, ethnicity is a major risk factor for GCA. The highest incidence figures are found in Scandinavian countries and among Americans of Scandinavian descent. The annual incidence of GCA in Olmsted County, Minnesota is 17 per 100,000 persons over the age of 50, similar to that in Scandinavian countries [3]. This similarity probably reflects shared genetic risk factors, as many of the inhabitants of Olmsted County during the period of study were descended from Scandinavians and northern Europeans. In Southern Europe and Mediterranean countries incidence rates are lower, with less than 10 per 100,000 persons over the age of 50 [5,7,8]. GCA is unusual in Latinos, Asians, and Arabs, though formal data on these populations are scant. The disease is decidedly uncommon in African Americans [9].

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Literature review current through: Oct 2017. | This topic last updated: Jun 08, 2017.
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  1. González-Gay MA, García-Porrúa C. Systemic vasculitis in adults in northwestern Spain, 1988-1997. Clinical and epidemiologic aspects. Medicine (Baltimore) 1999; 78:292.
  2. Crowson CS, Matteson EL, Myasoedova E, et al. The lifetime risk of adult-onset rheumatoid arthritis and other inflammatory autoimmune rheumatic diseases. Arthritis Rheum 2011; 63:633.
  3. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ, et al. Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum 2009; 61:1454.
  4. Salvarani C, Crowson CS, O'Fallon WM, et al. Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a fifty-year period. Arthritis Rheum 2004; 51:264.
  5. Gonzalez-Gay MA, Miranda-Filloy JA, Lopez-Diaz MJ, et al. Giant cell arteritis in northwestern Spain: a 25-year epidemiologic study. Medicine (Baltimore) 2007; 86:61.
  6. Kermani TA, Schäfer VS, Crowson CS, et al. Increase in age at onset of giant cell arteritis: a population-based study. Ann Rheum Dis 2010; 69:780.
  7. Bas-Lando M, Breuer GS, Berkun Y, et al. The incidence of giant cell arteritis in Jerusalem over a 25-year period: annual and seasonal fluctuations. Clin Exp Rheumatol 2007; 25:S15.
  8. Salvarani C, Macchioni P, Zizzi F, et al. Epidemiologic and immunogenetic aspects of polymyalgia rheumatica and giant cell arteritis in northern Italy. Arthritis Rheum 1991; 34:351.
  9. Smith CA, Fidler WJ, Pinals RS. The epidemiology of giant cell arteritis. Report of a ten-year study in Shelby County, Tennessee. Arthritis Rheum 1983; 26:1214.
  10. Ostberg G. An arteritis with special reference to polymyalgia arteritica. Acta Pathol Microbiol Scand Suppl 1973; 237:Suppl 237:1.
  11. Liozon E, Ouattara B, Rhaiem K, et al. Familial aggregation in giant cell arteritis and polymyalgia rheumatica: a comprehensive literature review including 4 new families. Clin Exp Rheumatol 2009; 27:S89.
  12. González-Gay MA, Blanco R, Abraira V, et al. Giant cell arteritis in Lugo, Spain, is associated with low longterm mortality. J Rheumatol 1997; 24:2171.
  13. Gran JT, Myklebust G, Wilsgaard T, Jacobsen BK. Survival in polymyalgia rheumatica and temporal arteritis: a study of 398 cases and matched population controls. Rheumatology (Oxford) 2001; 40:1238.
  14. Kermani TA, Warrington KJ, Crowson CS, et al. Large-vessel involvement in giant cell arteritis: a population-based cohort study of the incidence-trends and prognosis. Ann Rheum Dis 2013; 72:1989.
  15. Gonzalez-Gay MA, Barros S, Lopez-Diaz MJ, et al. Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients. Medicine (Baltimore) 2005; 84:269.
  16. Calamia KT, Hunder GG. Giant cell arteritis (temporal arteritis) presenting as fever of undetermined origin. Arthritis Rheum 1981; 24:1414.
  17. Knockaert DC, Vanneste LJ, Bobbaers HJ. Fever of unknown origin in elderly patients. J Am Geriatr Soc 1993; 41:1187.
  18. Jones JG. Clinical features of giant cell arteritis. Baillieres Clin Rheumatol 1991; 5:413.
  19. Myklebust G, Gran JT. A prospective study of 287 patients with polymyalgia rheumatica and temporal arteritis: clinical and laboratory manifestations at onset of disease and at the time of diagnosis. Br J Rheumatol 1996; 35:1161.
  20. Gabriel SE, O'Fallon WM, Achkar AA, et al. The use of clinical characteristics to predict the results of temporal artery biopsy among patients with suspected giant cell arteritis. J Rheumatol 1995; 22:93.
  21. Aiello PD, Trautmann JC, McPhee TJ, et al. Visual prognosis in giant cell arteritis. Ophthalmology 1993; 100:550.
  22. Font C, Cid MC, Coll-Vinent B, et al. Clinical features in patients with permanent visual loss due to biopsy-proven giant cell arteritis. Br J Rheumatol 1997; 36:251.
  23. González-Gay MA, García-Porrúa C, Llorca J, et al. Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients. Medicine (Baltimore) 2000; 79:283.
  24. Liozon E, Herrmann F, Ly K, et al. Risk factors for visual loss in giant cell (temporal) arteritis: a prospective study of 174 patients. Am J Med 2001; 111:211.
  25. Nesher G, Berkun Y, Mates M, et al. Risk factors for cranial ischemic complications in giant cell arteritis. Medicine (Baltimore) 2004; 83:114.
  26. Salvarani C, Cimino L, Macchioni P, et al. Risk factors for visual loss in an Italian population-based cohort of patients with giant cell arteritis. Arthritis Rheum 2005; 53:293.
  27. Danesh-Meyer H, Savino PJ, Gamble GG. Poor prognosis of visual outcome after visual loss from giant cell arteritis. Ophthalmology 2005; 112:1098.
  28. Jonasson F, Cullen JF, Elton RA. Temporal arteritis. A 14-year epidemiological, clinical and prognostic study. Scott Med J 1979; 24:111.
  29. Cid MC, Font C, Oristrell J, et al. Association between strong inflammatory response and low risk of developing visual loss and other cranial ischemic complications in giant cell (temporal) arteritis. Arthritis Rheum 1998; 41:26.
  30. Miller NR. Visual manifestations of temporal arteritis. Rheum Dis Clin North Am 2001; 27:781.
  31. Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis. Am J Ophthalmol 1998; 125:509.
  32. Guyer DR, Miller NR, Auer CL, Fine SL. The risk of cerebrovascular and cardiovascular disease in patients with anterior ischemic optic neuropathy. Arch Ophthalmol 1985; 103:1136.
  33. Hayreh SS. Ocular vascular occlusive disorders: natural history of visual outcome. Prog Retin Eye Res 2014; 41:1.
  34. Mohan K, Gupta A, Jain IS, Banerjee CK. Bilateral central retinal artery occlusion in occult temporal arteritis. J Clin Neuroophthalmol 1989; 9:270.
  35. Sadda SR, Nee M, Miller NR, et al. Clinical spectrum of posterior ischemic optic neuropathy. Am J Ophthalmol 2001; 132:743.
  36. Caselli RJ, Hunder GG. Neurologic complications of giant cell (temporal) arteritis. Semin Neurol 1994; 14:349.
  37. Smetana GW, Shmerling RH. Does this patient have temporal arteritis? JAMA 2002; 287:92.
  38. 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 1972; 27:378.
  39. Barricks ME, Traviesa DB, Glaser JS, Levy IS. Ophthalmoplegia in cranial arteritis. Brain 1977; 100:209.
  40. Razavi M, Jones RD, Manzel K, et al. Steroid-responsive charles bonnet syndrome in temporal arteritis. J Neuropsychiatry Clin Neurosci 2004; 16:505.
  41. Salvarani C, Hunder GG. Musculoskeletal manifestations in a population-based cohort of patients with giant cell arteritis. Arthritis Rheum 1999; 42:1259.
  42. Blockmans D, de Ceuninck L, Vanderschueren S, et al. Repetitive 18F-fluorodeoxyglucose positron emission tomography in giant cell arteritis: a prospective study of 35 patients. Arthritis Rheum 2006; 55:131.
  43. Prieto-González S, Arguis P, García-Martínez A, et al. Large vessel involvement in biopsy-proven giant cell arteritis: prospective study in 40 newly diagnosed patients using CT angiography. Ann Rheum Dis 2012; 71:1170.
  44. Schmidt WA, Seifert A, Gromnica-Ihle E, et al. Ultrasound of proximal upper extremity arteries to increase the diagnostic yield in large-vessel giant cell arteritis. Rheumatology (Oxford) 2008; 47:96.
  45. Aschwanden M, Kesten F, Stern M, et al. Vascular involvement in patients with giant cell arteritis determined by duplex sonography of 2x11 arterial regions. Ann Rheum Dis 2010; 69:1356.
  46. Ghinoi A, Pipitone N, Nicolini A, et al. Large-vessel involvement in recent-onset giant cell arteritis: a case-control colour-Doppler sonography study. Rheumatology (Oxford) 2012; 51:730.
  47. Brack A, Martinez-Taboada V, Stanson A, et al. Disease pattern in cranial and large-vessel giant cell arteritis. Arthritis Rheum 1999; 42:311.
  48. Mackie SL, Hensor EM, Morgan AW, Pease CT. Should I send my patient with previous giant cell arteritis for imaging of the thoracic aorta? A systematic literature review and meta-analysis. Ann Rheum Dis 2014; 73:143.
  49. Nuenninghoff DM, Hunder GG, Christianson TJ, et al. Incidence and predictors of large-artery complication (aortic aneurysm, aortic dissection, and/or large-artery stenosis) in patients with giant cell arteritis: a population-based study over 50 years. Arthritis Rheum 2003; 48:3522.
  50. Gonzalez-Gay MA, Garcia-Porrua C, Piñeiro A, et al. Aortic aneurysm and dissection in patients with biopsy-proven giant cell arteritis from northwestern Spain: a population-based study. Medicine (Baltimore) 2004; 83:335.
  51. García-Martínez A, Hernández-Rodríguez J, Arguis P, et al. Development of aortic aneurysm/dilatation during the followup of patients with giant cell arteritis: a cross-sectional screening of fifty-four prospectively followed patients. Arthritis Rheum 2008; 59:422.
  52. Evans JM, O'Fallon WM, Hunder GG. Increased incidence of aortic aneurysm and dissection in giant cell (temporal) arteritis. A population-based study. Ann Intern Med 1995; 122:502.
  53. Robson JC, Kiran A, Maskell J, et al. The relative risk of aortic aneurysm in patients with giant cell arteritis compared with the general population of the UK. Ann Rheum Dis 2015; 74:129.
  54. Evans JM, Bowles CA, Bjornsson J, et al. Thoracic aortic aneurysm and rupture in giant cell arteritis. A descriptive study of 41 cases. Arthritis Rheum 1994; 37:1539.
  55. Stone JH, Khosroshahi A, Deshpande V, Stone JR. IgG4-related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases. Arthritis Care Res (Hoboken) 2010; 62:316.
  56. Schmidt J, Sunesen K, Kornum JB, et al. Predictors for pathologically confirmed aortitis after resection of the ascending aorta: a 12-year Danish nationwide population-based cross-sectional study. Arthritis Res Ther 2011; 13:R87.
  57. Rojo-Leyva F, Ratliff NB, Cosgrove DM 3rd, Hoffman GS. Study of 52 patients with idiopathic aortitis from a cohort of 1,204 surgical cases. Arthritis Rheum 2000; 43:901.
  58. Kerr LD, Chang YJ, Spiera H, Fallon JT. Occult active giant cell aortitis necessitating surgical repair. J Thorac Cardiovasc Surg 2000; 120:813.
  59. Miller DV, Isotalo PA, Weyand CM, et al. Surgical pathology of noninfectious ascending aortitis: a study of 45 cases with emphasis on an isolated variant. Am J Surg Pathol 2006; 30:1150.
  60. Liang KP, Chowdhary VR, Michet CJ, et al. Noninfectious ascending aortitis: a case series of 64 patients. J Rheumatol 2009; 36:2290.
  61. Wang H, Smith RN, Spooner AE, et al. Giant cell aortitis of the ascending aorta without signs or symptoms of systemic vasculitis is associated with elevated risk of distal aortic events. Arthritis Rheum 2012; 64:317.
  62. Klein RG, Hunder GG, Stanson AW, Sheps SG. Large artery involvement in giant cell (temporal) arteritis. Ann Intern Med 1975; 83:806.
  63. Kermani TA, Matteson EL, Hunder GG, Warrington KJ. Symptomatic lower extremity vasculitis in giant cell arteritis: a case series. J Rheumatol 2009; 36:2277.
  64. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Gomez-Acebo I, et al. Strokes at time of disease diagnosis in a series of 287 patients with biopsy-proven giant cell arteritis. Medicine (Baltimore) 2009; 88:227.
  65. Salvarani C, Della Bella C, Cimino L, et al. Risk factors for severe cranial ischaemic events in an Italian population-based cohort of patients with giant cell arteritis. Rheumatology (Oxford) 2009; 48:250.
  66. Rüegg S, Engelter S, Jeanneret C, et al. Bilateral vertebral artery occlusion resulting from giant cell arteritis: report of 3 cases and review of the literature. Medicine (Baltimore) 2003; 82:1.
  67. Wiszniewska M, Devuyst G, Bogousslavsky J. Giant cell arteritis as a cause of first-ever stroke. Cerebrovasc Dis 2007; 24:226.
  68. Salvarani C, Giannini C, Miller DV, Hunder G. Giant cell arteritis: Involvement of intracranial arteries. Arthritis Rheum 2006; 55:985.
  69. Caselli RJ, Hunder GG. Neurologic aspects of giant cell (temporal) arteritis. Rheum Dis Clin North Am 1993; 19:941.
  70. Caselli RJ, Daube JR, Hunder GG, Whisnant JP. Peripheral neuropathic syndromes in giant cell (temporal) arteritis. Neurology 1988; 38:685.
  71. Larson TS, Hall S, Hepper NG, Hunder GG. Respiratory tract symptoms as a clue to giant cell arteritis. Ann Intern Med 1984; 101:594.
  72. Sendino A, Barbado FJ, González-Anglada I, et al. Temporal arteritis: a form of systemic panarteritis. Ann Rheum Dis 1992; 51:1082.
  73. Helfrich DJ, Mulhern LM, Luparello FJ, Smith W Jr. Giant cell arteritis of the tongue presenting as macroglossia. J Rheumatol 1988; 15:1026.
  74. Tsianakas A, Ehrchen JM, Presser D, et al. Scalp necrosis in giant cell arteritis: case report and review of the relevance of this cutaneous sign of large-vessel vasculitis. J Am Acad Dermatol 2009; 61:701.
  75. Brodmann M, Dorr A, Hafner F, et al. Tongue necrosis as first symptom of giant cell arteritis (GCA). Clin Rheumatol 2009; 28 Suppl 1:S47.
  76. Lee CC, Su WW, Hunder GG. Dysarthria associated with giant cell arteritis. J Rheumatol 1999; 26:931.
  77. Amor-Dorado JC, Llorca J, Garcia-Porrua C, et al. Audiovestibular manifestations in giant cell arteritis: a prospective study. Medicine (Baltimore) 2003; 82:13.
  78. Hernández-Rodríguez J, Tan CD, Molloy ES, et al. Vasculitis involving the breast: a clinical and histopathologic analysis of 34 patients. Medicine (Baltimore) 2008; 87:61.
  79. Bajocchi G, Zamorani G, Cavazza A, et al. Giant-cell arteritis of the female genital tract associated with occult temporal arteritis and FDG-PET evidence of large-vessel vasculitis. Clin Exp Rheumatol 2007; 25:S36.
  80. Scola CJ, Li C, Upchurch KS. Mesenteric involvement in giant cell arteritis. An underrecognized complication? Analysis of a case series with clinicoanatomic correlation. Medicine (Baltimore) 2008; 87:45.
  81. Bablekos GD, Michaelides SA, Karachalios GN, et al. Pericardial involvement as an atypical manifestation of giant cell arteritis: report of a clinical case and literature review. Am J Med Sci 2006; 332:198.
  82. Weiss LM, Gonzalez E, Miller SB, Agudelo CA. Severe anemia as the presenting manifestation of giant cell arteritis. Arthritis Rheum 1995; 38:434.
  83. Foroozan R, Danesh-Meyer H, Savino PJ, et al. Thrombocytosis in patients with biopsy-proven giant cell arteritis. Ophthalmology 2002; 109:1267.
  84. Gonzalez-Gay MA, Lopez-Diaz MJ, Barros S, et al. Giant cell arteritis: laboratory tests at the time of diagnosis in a series of 240 patients. Medicine (Baltimore) 2005; 84:277.
  85. Salvarani C, Hunder GG. Giant cell arteritis with low erythrocyte sedimentation rate: frequency of occurence in a population-based study. Arthritis Rheum 2001; 45:140.
  86. Liozon E, Jauberteau-Marchan MO, Ly K, et al. Giant cell arteritis with a low erythrocyte sedimentation rate: comments on the article by Salvarani and Hunder. Arthritis Rheum 2002; 47:692.
  87. Kermani TA, Schmidt J, Crowson CS, et al. Utility of erythrocyte sedimentation rate and C-reactive protein for the diagnosis of giant cell arteritis. Semin Arthritis Rheum 2012; 41:866.
  88. Roche NE, Fulbright JW, Wagner AD, et al. Correlation of interleukin-6 production and disease activity in polymyalgia rheumatica and giant cell arteritis. Arthritis Rheum 1993; 36:1286.
  89. Weyand CM, Fulbright JW, Hunder GG, et al. Treatment of giant cell arteritis: interleukin-6 as a biologic marker of disease activity. Arthritis Rheum 2000; 43:1041.