Official reprint from UpToDate®
www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Clinical features and diagnosis of Takayasu arteritis

Peter A Merkel, MD, MPH
Section Editor
Eric L Matteson, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


Takayasu arteritis (TAK) is classified as a large-vessel vasculitis because it primarily affects the aorta and its primary branches. It also shares some histologic and clinical features with giant cell (temporal) arteritis (GCA), the other major large-vessel vasculitis. Patients may present initially with constitutional symptoms but later develop symptoms associated with vascular damage.

The pathogenesis, pathology, clinical manifestations, and diagnosis of TAK will be reviewed here. The treatment of this disorder is discussed separately (see "Treatment of Takayasu arteritis"). Overviews of the vasculitides in children and in adults are also discussed elsewhere. (See "Vasculitis in children: Classification and incidence" and "Overview of and approach to the vasculitides in adults".)


Women are affected in 80 to 90 percent of cases, with an age of onset that is usually between 10 and 40 years [1,2]. It has a worldwide distribution, with the greatest prevalence in Asia [3-5]. In Japan, it has been estimated that 150 new cases occur each year [6]; by comparison, the incidence is one to three new cases per year per million population in the United States and Europe [2].


The pathogenesis of Takayasu arteritis (TAK) is poorly understood. Cell-mediated mechanisms are thought to be of primary importance and may be similar to those in giant cell arteritis (GCA) [7]. (See "Pathogenesis of giant cell (temporal) arteritis".)

Immunohistopathologic examination has shown that the infiltrating cells in aortic tissue mainly consist of killer cells, especially gamma delta T lymphocytes [8]. These cells may cause vascular injury by releasing large amounts of the cytolytic compound perforin. Recognition of heat shock protein-65 might facilitate recognition and adhesion of the infiltrating cells. In another report, the T cell receptors on the infiltrating T cells had a restricted repertoire, suggesting that a specific but as yet unidentified antigen in aortic tissue might be targeted [9]. This change was not seen in atherosclerotic aortic aneurysms.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Dec 2017. | This topic last updated: Jun 05, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2018 UpToDate, Inc.
  1. Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, et al. Takayasu's arteritis. Clinical study of 107 cases. Am Heart J 1977; 93:94.
  2. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33:1129.
  3. Dabague J, Reyes PA. Takayasu arteritis in Mexico: a 38-year clinical perspective through literature review. Int J Cardiol 1996; 54 Suppl:S103.
  4. Hall S, Barr W, Lie JT, et al. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore) 1985; 64:89.
  5. Ishikawa K. Natural history and classification of occlusive thromboaortopathy (Takayasu's disease). Circulation 1978; 57:27.
  6. Koide K. Takayasu arteritis in Japan. Heart Vessels Suppl 1992; 7:48.
  7. Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med 2003; 349:160.
  8. Seko Y, Minota S, Kawasaki A, et al. Perforin-secreting killer cell infiltration and expression of a 65-kD heat-shock protein in aortic tissue of patients with Takayasu's arteritis. J Clin Invest 1994; 93:750.
  9. Seko Y, Sato O, Takagi A, et al. Restricted usage of T-cell receptor Valpha-Vbeta genes in infiltrating cells in aortic tissue of patients with Takayasu's arteritis. Circulation 1996; 93:1788.
  10. Cid MC, Font C, Coll-Vinent B, Grau JM. Large vessel vasculitides. Curr Opin Rheumatol 1998; 10:18.
  11. Yamada I, Shibuya H, Matsubara O, et al. Pulmonary artery disease in Takayasu's arteritis: angiographic findings. AJR Am J Roentgenol 1992; 159:263.
  12. Mason JC. Takayasu arteritis--advances in diagnosis and management. Nat Rev Rheumatol 2010; 6:406.
  13. Serra R, Butrico L, Fugetto F, et al. Updates in Pathophysiology, Diagnosis and Management of Takayasu Arteritis. Ann Vasc Surg 2016; 35:210.
  14. Rodríguez-Pla A, de Miguel G, López-Contreras J, et al. Bilateral blindness in Takayasu's disease. Scand J Rheumatol 1996; 25:394.
  15. Nakabayashi K, Kurata N, Nangi N, et al. Pulmonary artery involvement as first manifestation in three cases of Takayasu arteritis. Int J Cardiol 1996; 54 Suppl:S177.
  16. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med 1994; 120:919.
  17. Yoneda S, Nukada T, Tada K, et al. Subclavian steal in Takayasu's arteritis. A hemodynamic study by means of ultrasonic Doppler flowmetry. Stroke 1977; 8:264.
  18. Grayson PC, Tomasson G, Cuthbertson D, et al. Association of vascular physical examination findings and arteriographic lesions in large vessel vasculitis. J Rheumatol 2012; 39:303.
  19. Hata A, Numano F. Magnetic resonance imaging of vascular changes in Takayasu arteritis. Int J Cardiol 1995; 52:45.
  20. Yamada I, Numano F, Suzuki S. Takayasu arteritis: evaluation with MR imaging. Radiology 1993; 188:89.
  21. Yamada I, Nakagawa T, Himeno Y, et al. Takayasu arteritis: evaluation of the thoracic aorta with CT angiography. Radiology 1998; 209:103.
  22. Paul JF, Hernigou A, Lefebvre C, et al. Electron beam CT features of the pulmonary artery in Takayasu's arteritis. AJR Am J Roentgenol 1999; 173:89.
  23. Kissin EY, Merkel PA. Diagnostic imaging in Takayasu arteritis. Curr Opin Rheumatol 2004; 16:31.
  24. Keenan NG, Mason JC, Maceira A, et al. Integrated cardiac and vascular assessment in Takayasu arteritis by cardiovascular magnetic resonance. Arthritis Rheum 2009; 60:3501.
  25. Fassbender HG. Pathology and pathobiology of rheumatic diseases, Second Edition, Springer-Verlag, Berlin 2002. p.304.
  26. Nasu T. Takayasu's truncoarteritis. Pulseless disease or aortitis syndrome. Acta Pathol Jpn 1982; 32 Suppl 1:117.
  27. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
  28. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37:187.
  29. Ishikawa K. Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu's arteriopathy. J Am Coll Cardiol 1988; 12:964.
  30. Sharma BK, Jain S, Suri S, Numano F. Diagnostic criteria for Takayasu arteritis. Int J Cardiol 1996; 54 Suppl:S141.
  31. Craven A, Robson J, Ponte C, et al. ACR/EULAR-endorsed study to develop Diagnostic and Classification Criteria for Vasculitis (DCVAS). Clin Exp Nephrol 2013; 17:619.
  32. Luqmani RA, Suppiah R, Grayson PC, et al. Nomenclature and classification of vasculitis - update on the ACR/EULAR diagnosis and classification of vasculitis study (DCVAS). Clin Exp Immunol 2011; 164 Suppl 1:11.
  33. Hunder GG. Giant cell arteritis and polymyalgia rheumatica. Med Clin North Am 1997; 81:195.
  34. Michel BA, Arend WP, Hunder GG. Clinical differentiation between giant cell (temporal) arteritis and Takayasu's arteritis. J Rheumatol 1996; 23:106.
  35. Grayson PC, Maksimowicz-McKinnon K, Clark TM, et al. Distribution of arterial lesions in Takayasu's arteritis and giant cell arteritis. Ann Rheum Dis 2012; 71:1329.
  36. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Takayasu arteritis and giant cell arteritis: a spectrum within the same disease? Medicine (Baltimore) 2009; 88:221.