Advertisement

Current Rheumatology Reports

, Volume 12, Issue 6, pp 436–442 | Cite as

Giant Cell Arteritis: Epidemiology, Diagnosis, and Management

  • Miguel A. Gonzalez-GayEmail author
  • Cristina Martinez-Dubois
  • Mario Agudo
  • Orlando Pompei
  • Ricardo Blanco
  • Javier Llorca
Article

Abstract

Giant cell arteritis (GCA), also called temporal arteritis, is a vasculitis that affects large and middle-sized blood vessels—with predisposition to the involvement of cranial arteries derived from the carotid artery—in individuals older than 50 years of age. Familial aggregation of GCA has been observed. Incidence of GCA is higher in white individuals than those of other ethnicities, particularly those of Scandinavian background. A temporal artery biopsy is the gold standard test for the diagnosis of GCA. Several imaging modalities, in particular ultrasonography, are useful in the diagnosis of GCA. Corticosteroids are the cornerstone of treatment in GCA. Alternative, steroid-sparing drugs, particularly methotrexate, should be considered in GCA patients with severe corticosteroid-related side effects and/or in those who require prolonged corticosteroid therapy due to relapses of the disease.

Keywords

Giant cell arteritis Incidence Temporal artery biopsy Ultrasonography Corticosteroids 

Notes

Disclosure

No potential conflicts of interest relevant to this article were reported.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    •• Salvarani C, Cantini F, Hunder GG: Polymyalgia rheumatica and giant-cell arteritis. Lancet 2008, 372:234–245. This review article describes the main clinical findings regarding GCA. The authors also discussed the use of corticosteroids, the cornerstone therapy for GCA.CrossRefPubMedGoogle Scholar
  2. 2.
    •• Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ, et al.: Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum 2009, 61:1454–1461. This review article assessed current information on the epidemiology of GCA and PMR. Different issues, including etiology, incidence, influence of environmental factors and infectious agents, implication of genetic factors, influence of traditional cardiovascular risk factors, the role of biopsy in the incidence of GCA, and the prognosis of GCA and PMR, were discussed.CrossRefPubMedGoogle Scholar
  3. 3.
    • 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(Suppl 52):S89–S94. This was a survey on familial aggregation of GCA and PMR showing data that suggest the presence of a genetic predisposition for these conditions.PubMedGoogle Scholar
  4. 4.
    González-Gay MA, Amoli MM, Garcia-Porrua C, Ollier WE: Genetic markers of disease susceptibility and severity in giant cell arteritis and polymyalgia rheumatica. Semin Arthritis Rheum 2003, 33:38–48.CrossRefPubMedGoogle Scholar
  5. 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, 86:61–88.CrossRefGoogle Scholar
  6. 6.
    Gonzalez-Gay MA, Blanco R, Sanchez-Andrade A, Vazquez-Caruncho M: Giant cell arteritis in Lugo, Spain: a more frequent disease with fewer classic features. J Rheumatol 1997, 24:2166–2170.PubMedGoogle Scholar
  7. 7.
    • Lopez-Diaz MJ, Llorca J, Gonzalez-Juanatey C, et al.: Implication of the age in the clinical spectrum of giant cell arteritis. Clin Exp Rheumatol 2008, 26(Suppl 49):S16–S22. Some differences in the clinical spectrum of manifestations of GCA seem to occur according to patient age at onset of the disease.PubMedGoogle Scholar
  8. 8.
    • 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, 69:780–781. Data from Olmsted County, MN, indicated an increase in age at GCA diagnosis that seemed to be related to a significant increase in the incidence of GCA in individuals 70 years of age and older.CrossRefPubMedGoogle Scholar
  9. 9.
    Salvarani C, Gabriel SE, O’Fallon WM, Hunder GG: The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern. Ann Intern Med 1995, 123:192–194.PubMedGoogle Scholar
  10. 10.
    Petursdottir V, Johansson H, Nordborg E, Nordborg C: The epidemiology of biopsy-positive giant cell arteritis: special reference top cyclic fluctuations. Rheumatology (Oxford) 1999, 38:1208–1212.CrossRefGoogle Scholar
  11. 11.
    • Khalifa M, Karmani M, Jaafoura NG, et al.; Study Group of GCA in Tunisia: Epidemiological and clinical features of giant cell arteritis in Tunisia. Eur J Intern Med 2009, 20:208–212. In Tunisia, the clinical spectrum of GCA manifestations is similar to that reported in areas in which GCA is more prevalent.CrossRefPubMedGoogle Scholar
  12. 12.
    • Pamuk ON, Dönmez S, Karahan B, et al.: Giant cell arteritis and polymyalgia rheumatica in northwestern Turkey: clinical features and epidemiological data. Clin Exp Rheumatol 2009, 27:830–833. In Turkey, the annual incidence rate of GCA is very low.PubMedGoogle Scholar
  13. 13.
    • Mader TH, Werner RP, Chamberlain DG, Doornbos D: Giant cell arteritis in Alaska natives. Can J Ophthalmol 2009, 44:53–56. Incidence of biopsy-proven GCA among Alaska natives is also very low.CrossRefPubMedGoogle Scholar
  14. 14.
    Gonzalez-Gay M: The diagnosis and management of patients with giant cell arteritis. J Rheumatol 2005, 32:1186–1188.PubMedGoogle Scholar
  15. 15.
    Taylor-Gjevre R, Vo M, Shukla D, Resch L: Temporal artery biopsy for giant cell arteritis. J Rheumatol 2005, 32:1279–1282.PubMedGoogle Scholar
  16. 16.
    Mahr A, Saba M, Kambouchner M, et al.: Temporal artery biopsy for diagnosing giant cell arteritis: the longer, the better? Ann Rheum Dis 2006, 65:826–828.CrossRefPubMedGoogle Scholar
  17. 17.
    •• Breuer GS, Nesher G, Nesher R: Rate of discordant findings in bilateral temporal artery biopsy to diagnose giant cell arteritis. J Rheumatol 2009, 36:794–796. This interesting study shows that bilateral TABs increase the diagnostic sensitivity of the procedure by up to 12.7% compared with unilateral biopsies.CrossRefPubMedGoogle Scholar
  18. 18.
    •• Breuer GS, Nesher R, Nesher G: Effect of biopsy length on the rate of positive temporal artery biopsies. Clin Exp Rheumatol 2009, 27(Suppl 52):S10–S13. TAB with postfixation length shorter than 5 mm carries an increased biopsy-negative rate. Therefore, longer TAB length is required for accurate diagnosis. Increasing postfixation TAB length beyond 20 mm may further increase the rate of positive biopsies.PubMedGoogle Scholar
  19. 19.
    •• Marí B, Monteagudo M, Bustamante E, et al.: Analysis of temporal artery biopsies in an 18-year period at a community hospital. Eur J Intern Med 2009, 20:533–536. Headache, jaw claudication, and abnormal temporal artery on palpation were the best positive predictors of positive TAB in a community hospital. Also, a significantly increased frequency of severe visual ischemic complications was found in the group of biopsy-proven GCA patients compared with GCA patients with a negative TAB.CrossRefPubMedGoogle Scholar
  20. 20.
    Gonzalez-Gay MA, Garcia-Porrua C, Llorca J, et al.: Biopsy-negative giant cell arteritis: clinical spectrum and predictive factors for positive temporal artery biopsy. Semin Arthritis Rheum 2001, 30:249–256.CrossRefPubMedGoogle Scholar
  21. 21.
    Duhaut P, Pinède L, Bornet H, et al.: Biopsy proven and biopsy negative temporal arteritis: differences in clinical spectrum at the onset of the disease. Groupe de Recherche sur l’Artérite à Cellules Géantes. Ann Rheum Dis 1999, 58:335–341.Google Scholar
  22. 22.
    •• Pipitone N, Versari A, Salvarani C: Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford) 2008, 47:403–408. This is an extremely interesting review article addressing the role of imaging techniques in the diagnosis and follow-up of patients with large vessel vasculitis.CrossRefGoogle Scholar
  23. 23.
    Schmidt WA, Kraft HE, Vorpahl K, et al.: Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med 1997, 337:1336–1342.CrossRefPubMedGoogle Scholar
  24. 24.
    •• Arida A, Kyprianou M, Kanakis M, Sfikakis PP: The diagnostic value of ultrasonography-derived edema of the temporal artery wall in giant cell arteritis: a second meta-analysis. BMC Musculoskelet Disord 2010, 11:44. This was a very interesting meta-analysis. The study confirmed that the halo sign in US is useful in making a diagnosis of GCA.CrossRefPubMedGoogle Scholar
  25. 25.
    •• Schmidt WA, Krause A, Schicke B, et al.: Do temporal artery duplex ultrasound findings correlate with ophthalmic complications in giant cell arteritis? Rheumatology (Oxford) 2009, 48:383–385. This study showed that findings of temporal artery US do not seem to correlate with eye complications in patients with GCA.CrossRefGoogle Scholar
  26. 26.
    • Pérez López J, Solans Laqué R, Bosch Gil JA, et al.: Colour-duplex ultrasonography of the temporal and ophthalmic arteries in the diagnosis and follow-up of giant cell arteritis. Clin Exp Rheumatol 2009, 27(Suppl 52):S77–S82. Color Doppler US of the ophthalmic arteries is not useful in making a diagnosis of GCA.PubMedGoogle Scholar
  27. 27.
    •• Martínez-Valle F, Solans-Laqué R, Bosch-Gil J, et al.: Aortic involvement in giant cell arteritis. Autoimmun Rev 2010, 9:521–524. The authors emphasize that aortic involvement probably has been underestimated, and its incidence may be more frequent than suspected in patients with GCA. Therefore, evaluation of patients with imaging techniques such as magnetic resonance angiography and PET may be useful to establish the clinical impact of extracranial involvement caused by this vasculitis.CrossRefPubMedGoogle Scholar
  28. 28.
    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, 55:131–137.CrossRefPubMedGoogle Scholar
  29. 29.
    Gonzalez-Gay MA: Giant cell arteritis and polymyalgia rheumatica: two different but often overlapping conditions. Semin Arthritis Rheum 2004, 33:289–293.CrossRefPubMedGoogle Scholar
  30. 30.
    Gonzalez-Gay MA, Garcia-Porrua C, Amor-Dorado JC, Llorca J: Giant cell arteritis without clinically evident vascular involvement in a defined population. Arthritis Rheum 2004, 51:274–277.CrossRefPubMedGoogle Scholar
  31. 31.
    • Henes JC, Müller M, Krieger J, et al.: [18F] FDG-PET/CT as a new and sensitive imaging method for the diagnosis of large vessel vasculitis. Clin Exp Rheumatol 2008, 26(Suppl 49):S47–S52. Unlike Doppler US, PET/CT provides information on disease activity and vessel morphology as well as the localization of the inflammatory process in the same session in patients with large vessel vasculitis.PubMedGoogle Scholar
  32. 32.
    •• Ghinoi A, Zuccoli G, Nicolini A, et al.: 1 T magnetic resonance imaging in the diagnosis of giant cell arteritis: comparison with ultrasonography and physical examination of temporal arteries. Clin Exp Rheumatol 2008, 26(Suppl 49):S76–S80. Temporal artery abnormalities on physical examination and the presence of a hypoechoic halo on US yielded higher sensitivity than 1 T MRI of the temporal artery.PubMedGoogle Scholar
  33. 33.
    •• Bley TA, Reinhard M, Hauenstein C, et al.: Comparison of duplex sonography and high-resolution magnetic resonance imaging in the diagnosis of giant cell (temporal) arteritis. Arthritis Rheum 2008, 58:2574–2578. In this study, the diagnostic power of high-resolution MRI and color-coded duplex US to detect GCA was similar. According to these results, both noninvasive techniques may have value in the evaluation of patients with suspected GCA.CrossRefPubMedGoogle Scholar
  34. 34.
    • Agard C, Barrier JH, Dupas B, et al.: Aortic involvement in recent-onset giant cell (temporal) arteritis: a case-control prospective study using helical aortic computed tomodensitometric scan. Arthritis Rheum 2008, 59:670–676. This study suggests that inflammatory aortic thickening, detected by computed tomodensitometric scan, occurs frequently at the time of diagnosis of GCA.CrossRefPubMedGoogle Scholar
  35. 35.
    • Mukhtyar C, Guillevin L, Cid MC, et al.: EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2009, 68:318–323. This article includes several recommendations for the management of patients with GCA.CrossRefPubMedGoogle Scholar
  36. 36.
    Caselli RJ, Hunder GG, Whisnant JP: Neurologic disease in biopsy-proven giant cell (temporal arteritis). Neurology 1988, 38:352–359.PubMedGoogle Scholar
  37. 37.
    Foroozan R, Deramo VA, Buono LM, et al.: Recovery of visual function in patients with biopsy-proven giant cell arteritis. Ophthalmology 2003, 110:539–542.CrossRefPubMedGoogle Scholar
  38. 38.
    Neff AG, Greifenstein EM: Giant cell arteritis update. Semin Ophthalmol 1999, 14:109–112.CrossRefPubMedGoogle Scholar
  39. 39.
    Gonzalez-Gay MA, Blanco R, Rodriguez-Valverde V, et al.: Permanent visual loss and cerebrovascular accidents in giant cell arteritis: predictors and response to treatment. Arthritis Rheum 1998, 41:1497–1504.CrossRefPubMedGoogle Scholar
  40. 40.
    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, 84:269–276.CrossRefGoogle Scholar
  41. 41.
    Hunder GG, Sheps SG, Allen GL, Joyce JW: Daily and alternate-day corticosteroid regimens in treatment of giant cell arteritis: comparison in a prospective study. Ann Intern Med 1975, 82:613–618.PubMedGoogle Scholar
  42. 42.
    Mazlumzadeh M, Hunder GG, Easley KA, et al.: Treatment of giant cell arteritis using induction therapy with high-dose glucocorticoids: a double-blind, placebo-controlled, randomized prospective clinical trial. Arthritis Rheum 2006, 54:3310–3318.CrossRefPubMedGoogle Scholar
  43. 43.
    Mahr AD, Jover JA, Spiera RF, et al.: Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum 2007, 56:2789–2797.CrossRefPubMedGoogle Scholar
  44. 44.
    Gonzalez-Gay MA, Piñeiro A, Gomez-Gigirey A, et al.: Influence of traditional risk factors of atherosclerosis in the development of severe ischemic complications in giant cell arteritis. Medicine (Baltimore) 2004, 83:342–347.CrossRefGoogle Scholar
  45. 45.
    Nesher G, Berkun Y, Mates M, et al.: Low-dose aspirin and prevention of cranial ischemic complications in giant cell arteritis. Arthritis Rheum 2004, 50:1332–1337.CrossRefPubMedGoogle Scholar
  46. 46.
    Lee MS, Smith SD, Galor A, Hoffman GS: Antiplatelet and anticoagulant therapy in patients with giant cell arteritis. Arthritis Rheum 2006, 54:3306–3309.CrossRefPubMedGoogle Scholar
  47. 47.
    Lopez-Diaz MJ, Llorca J, Gonzalez-Juanatey C, et al.: The erythrocyte sedimentation rate is associated with the development of visual complications in biopsy-proven giant cell arteritis. Semin Arthritis Rheum 2008, 38:116–123.CrossRefPubMedGoogle Scholar
  48. 48.
    Narváez J, Bernad B, Gómez-Vaquero C, et al.: Impact of antiplatelet therapy in the development of severe ischemic complications and in the outcome of patients with giant cell arteritis. Clin Exp Rheumatol 2008, 26(Suppl 49):S57–S62.PubMedGoogle Scholar
  49. 49.
    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, 48:250–253.CrossRefGoogle Scholar
  50. 50.
    • Berger CT, Wolbers M, Meyer P, et al.: High incidence of severe ischaemic complications in patients with giant cell arteritis irrespective of platelet count and size, and platelet inhibition. Rheumatology (Oxford) 2009, 48:258–261. In this study and those by Lopez-Diaz et al. [47], Narváez et al. [48], and Salvarani et al. [49], a reduction in the risk of severe ischemic complications following antiaggregation therapy was not confirmed. CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Miguel A. Gonzalez-Gay
    • 1
    Email author
  • Cristina Martinez-Dubois
    • 1
  • Mario Agudo
    • 1
  • Orlando Pompei
    • 1
  • Ricardo Blanco
    • 1
  • Javier Llorca
    • 1
  1. 1.Rheumatology DivisionHospital Universitario Marques de ValdecillaSantanderSpain

Personalised recommendations