Large-Vessel Vasculitides: Takayasu Arteritis and Giant Cell Arteritis

  • Giulio Cavalli
  • Giacomo De Luca
  • Lorenzo DagnaEmail author


Vasculitis is an inflammatory process affecting blood vessels. Based on the involvement of the endothelium or the wall of an artery or vein, the inflammatory process can cause thickening, weakening, stenosis, occlusion, aneurysmal dilation, and scarring of the vessel, limiting blood supply to tissues and leading to partial or complete organ failure. Vasculitides affecting large arteries include Takayasu arteritis (TA) and giant cell arteritis (GCA). TA is a rare disease, which is more commonly observed in parts of Asia and the Middle East and typically affects younger women. TA causes inflammation of the aorta and its main branches and may cause a broad spectrum of symptoms ranging from limb claudication to chest pain.

GCA is the most common form of vasculitis in adults. While also affecting the aorta and its branches, GCA exhibits a predilection for cranial arteries. Common symptoms of GCA include headache, jaw claudication during prolonged chewing, and visual disturbances or loss of vision. Both TA and GCA require treatment with long-term immunosuppression, which is usually effective at controlling disease manifestations in the short term. However, treatment toxicity and relapses of vasculitis are also common, and selected patients require a surgical approach.


  1. 1.
    Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33(8):1129.CrossRefGoogle Scholar
  2. 2.
    Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. NEJM. 2003;349(2):160.CrossRefGoogle Scholar
  3. 3.
    Mason JC. Takayasu arteritis--advances in diagnosis and management. Nat Rev Rheumatol. 2010;6(7):406–15.CrossRefGoogle Scholar
  4. 4.
    Serra R, et al. Updates in pathophysiology, diagnosis and management of Takayasu arteritis. Ann Vasc Surg. 2016;35:210–25.CrossRefGoogle Scholar
  5. 5.
    Kim E, et al. Takayasu arteritis: challenges in diagnosis and management. Heart. 2017;104(7):558–65.CrossRefGoogle Scholar
  6. 6.
    Alibaz-Oner F, et al. Update on Takayasu’s arteritis. Presse Med. 2015;44:e259–65.CrossRefGoogle Scholar
  7. 7.
    Barra L, et al. Imaging modalities for the diagnosis and disease activity assessment of Takayasu’s arteritis: a systematic review and meta-analysis. Autoimmun Rev. 2018;17(2):175–87.CrossRefGoogle Scholar
  8. 8.
    Keser G, et al. What is new in management of Takayasu arteritis? Presse Med. 2017;46:e229–35.CrossRefGoogle Scholar
  9. 9.
    Misra R, et al. Development and initial validation of the Indian Takayasu Clinical Activity Score (ITAS2010). Rheumatology (Oxford). 2013;52:1795–801.CrossRefGoogle Scholar
  10. 10.
    Abisror N, et al. Tocilizumab in refractory Takayasu arteritis: a case series and updated literature review. Autoimmun Rev. 2013;12:1143–9.CrossRefGoogle Scholar
  11. 11.
    Kazibudzki M, et al. New endovascular techniques for treatment of life-threatening Takayasu arteritis. Adv Interv Cardiol. 2016;12:171–4.CrossRefGoogle Scholar
  12. 12.
    Saadoun D, et al. Retrospective analysis of surgery versus endovascular intervention in Takayasu arteritis: a multicenter experience. Circulation. 2012;125:813–9.CrossRefGoogle Scholar
  13. 13.
    Matsuura K, et al. Surgical treatment of aortic regurgitation due to Takayasu arteritis: long-term morbidity and mortality. Circulation. 2005;112(24):3707.CrossRefGoogle Scholar
  14. 14.
    Schmidt J, et al. Diagnostic features, treatment, and outcomes of Takayasu arteritis in a US cohort of 126 patients. Mayo Clin Proc. 2013;88(8):822.CrossRefGoogle Scholar
  15. 15.
    Jennette JC, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11.CrossRefGoogle Scholar
  16. 16.
    Goodwin JS. Progress in gerontology: polymyalgia rheumatica and temporal arteritis. J Am Geriatr Soc. 1992;40(5):515–25.CrossRefGoogle Scholar
  17. 17.
    Gonzalez-Gay MA, et al. Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum. 2009;61(10):1454.CrossRefGoogle Scholar
  18. 18.
    Gonzalez-Gay MA, et al. Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients. Medicine (Baltimore). 2005;84(5):269.CrossRefGoogle Scholar
  19. 19.
    Buttgereit F, et al. Polymyalgia rheumatica and giant cell arteritis: a systematic review. JAMA. 2016;315(22):2442–58.CrossRefGoogle Scholar
  20. 20.
    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(11):1161–8.CrossRefGoogle Scholar
  21. 21.
    González-Gay MA, et al. Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients. Medicine (Baltimore). 2000;79(5):283–92.CrossRefGoogle Scholar
  22. 22.
    Salvarani C, et al. Risk factors for visual loss in an Italian population-based cohort of patients with giant cell arteritis. Arthritis Rheum. 2005;53(2):293–7.CrossRefGoogle Scholar
  23. 23.
    Soriano A, et al. Visual loss and other cranial ischaemic complications in giant cell arteritis. Nat Rev Rheumatol. 2017;13:476–84.CrossRefGoogle Scholar
  24. 24.
    Gonzalez-Gay MA, et al. Strokes at time of disease diagnosis in a series of 287 patients with biopsy-proven giant cell arteritis. Medicine (Baltimore). 2009;88(4):227–35.CrossRefGoogle Scholar
  25. 25.
    Lensen KD, et al. Extracranial giant cell arteritis. Neth J Med. 2016;74(5):182–92.PubMedGoogle Scholar
  26. 26.
    Nuenninghoff DM, 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;48:3522–31.CrossRefGoogle Scholar
  27. 27.
    Weyand CM, Goronzy JJ. Clinical practice: giant-cell arteritis and polymyalgia rheumatica. N Engl J Med. 2014;371(1):50–7.CrossRefGoogle Scholar
  28. 28.
    Gonzalez-Gay MA, et al. Giant cell arteritis: laboratory tests at the time of diagnosis in a series of 240 patients. Medicine (Baltimore). 2005;84(5):277.CrossRefGoogle Scholar
  29. 29.
    Niederkohr RD, Levin LA. A Bayesian analysis of the true sensitivity of a temporal artery biopsy. Invest Ophthalmol Vis Sci. 2007;48(2):675–80.CrossRefGoogle Scholar
  30. 30.
    Jakobsson K, et al. The effect of clinical features and glucocorticoids on biopsy findings in giant cell arteritis. BMC Musculoskelet Disord. 2016;17(1):363.CrossRefGoogle Scholar
  31. 31.
    Dejaco C, et al. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Ann Rheum Dis. 2018;77:636–43.CrossRefGoogle Scholar
  32. 32.
    Hunder GG, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990;33:1122–8.CrossRefGoogle Scholar
  33. 33.
    Dasgupta B, et al. BSR and BHPR guidelines for the management of giant cell arteritis. Rheumatology (Oxford). 2010;49:1594–7.CrossRefGoogle Scholar
  34. 34.
    Dejaco C, et al. Giant cell arteritis and polymyalgia rheumatica: current challenges and opportunities. Nat Rev Rheumatol. 2017;13:578–92.CrossRefGoogle Scholar
  35. 35.
    Hill CL, et al. Risk of mortality in patients with giant cell arteritis: a systematic review and meta-analysis. Semin Arthritis Rheum. 2017;46(4):513–9.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Giulio Cavalli
    • 1
  • Giacomo De Luca
    • 1
  • Lorenzo Dagna
    • 1
    Email author
  1. 1.Unit of Immunology, Rheumatology, Allergy and Rare DiseasesSan Raffaele Hospital and UniversityMilanItaly

Personalised recommendations