Highlights & Basics
- Takayasu arteritis is a vasculitis of large vessels that particularly affects the aorta and its primary branches.
- Generally more common in women and typically presents before the age of 40 years.
- Typical symptoms include limb claudication on exertion, chest pain, and systemic symptoms of weight loss, fatigue, low-grade fever, and myalgia.
- On examination, vascular bruits may be audible over the carotids, abdominal aorta, or subclavian vessels. Unequal blood pressures may be recorded between sides, and a murmur of aortic regurgitation may be heard if there is aortic root dilation.
- The diagnosis is usually made by vascular imaging.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Photomicrograph of the aorta from a patient with Takayasu arteritis demonstrates marked thickening of the intimal layer and inflammatory infiltrates in the media and laminar necrosis
Magnetic resonance angiogram of the aortic arch and major vessels showing occlusion of bilateral subclavian arteries; left common carotid artery has small diameter; proximal vertebral arteries are not identified
Computed tomography angiogram, with 3D reconstruction of the aortic arch and major vessels, showing proximal occlusion of the left subclavian artery and patent left vertebral artery distal to the occlusion (left vertebral steal syndrome)
Computed tomography angiogram, with 3D reconstruction of the aortic arch and major vessels, showing narrowing of the left common carotid artery and left subclavian artery
Computed tomography angiogram with 3D reconstruction showing bilateral renal artery stenosis
The final 2022 American College of Rheumatology/EULAR Classification Criteria for Takayasu arteritis
Citations
Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. 2003 Jul 10;349(2):160-9.[Abstract]
Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. 1995 Nov;21(4):1041-58.[Abstract]
Maz M, Chung SA, Abril A, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis. Arthritis Rheumatol. 2021 Aug;73(8):1349-65.[Abstract][Full Text]
Pugh D, Karabayas M, Basu N, et al. Large-vessel vasculitis. Nat Rev Dis Primers. 2022 Jan 6;7(1):93.[Abstract][Full Text]
Isselbacher EM, Preventza O, et al. 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022 Dec 13;80(24):e223-393.[Abstract]
Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis. Ann Rheum Dis. 2022 Dec;81(12):1654-60.[Abstract][Full Text]
1. Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. 2003 Jul 10;349(2):160-9.[Abstract]
2. Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. 1995 Nov;21(4):1041-58.[Abstract]
3. Maz M, Chung SA, Abril A, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis. Arthritis Rheumatol. 2021 Aug;73(8):1349-65.[Abstract][Full Text]
4. Pugh D, Karabayas M, Basu N, et al. Large-vessel vasculitis. Nat Rev Dis Primers. 2022 Jan 6;7(1):93.[Abstract][Full Text]
5. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 2013 Jan;65(1):1-11.[Abstract][Full Text]
6. Moriwaki R, Noda M, Yajima M, et al. Clinical manifestations of Takayasu arteritis in India and Japan - new classification of angiographic findings. Angiology. 1997 May;48(5):369-79.[Abstract]
7. Hall S, Barr W, Lie JT, et al. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore). 1985 Mar;64(2):89-99.[Abstract]
8. Maksimowicz-McKinnon K, Clark T, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007 Mar;56(3):1000-9.[Abstract][Full Text]
9. Numano F, Okawara M, Inomata H, et al. Takayasu's arteritis. Lancet. 2000 Sep 16;356(9234):1023-5.[Abstract]
10. Werfel T, Kuipers JG, Zeidler H, et al. Cutaneous manifestations of Takayasu arteritis. Acta Derm Venereol. 1996 Nov;76(6):496-7.[Abstract]
11. Waern AU, Anderson P, Hemmingsson A. Takayasu's arteritis: a hospital-region based study on occurrence, treatment and prognosis. Angiology. 1983 May;34(5):311-20.[Abstract]
12. Vieira M, Ochtrop MLG, Sztajnbok F, et al. The epidemiology of Takayasu arteritis in Rio de Janeiro, Brazil: a large population-based study. J Clin Rheumatol. 2023 Aug 1;29(5):e100-3.[Abstract]
13. Nasu T. Takayasu's truncoarteritis in Japan. A statistical observation of 76 autopsy cases. Pathol Microbiol (Basel). 1975;43(2-O):140-6.[Abstract]
14. Rutter M, Bowley J, Lanyon PC, et al. A systematic review and meta-analysis of the incidence rate of Takayasu arteritis. Rheumatology (Oxford). 2021 Nov 3;60(11):4982-90.[Abstract][Full Text]
15. Saruhan-Direskeneli G, Biçakçigil M, Yilmaz V, et al. Interleukin (IL)-12, IL-2, and IL-6 gene polymorphisms in Takayasu's arteritis from Turkey. Hum Immunol. 2006 Sep;67(9):735-40.[Abstract]
16. Kimura A, Kitamura H, Date Y, et al. Comprehensive analysis of HLA genes in Takayasu arteritis in Japan. Int J Cardiol. 1996 Aug;54 Suppl:S61-9.[Abstract]
17. Sahin N, Aksu K, Kamali S, et al. PTPN22 gene polymorphism in Takayasu's arteritis. Rheumatology (Oxford). 2008 May;47(5):634-5.[Abstract]
18. Misra DP, Chaurasia S, Misra R. Increased circulating Th17 cells, serum IL-17A, and IL-23 in Takayasu arteritis. Autoimmune Dis. 2016;2016:7841718.[Abstract][Full Text]
19. Clifford A, Hoffman GS. Recent advances in the medical management of Takayasu arteritis: an update on use of biologic therapies. Curr Opin Rheumatol. 2014 Jan;26(1):7-15.[Abstract]
20. Nakaoka Y, Isobe M, Takei S, et al. Efficacy and safety of tocilizumab in patients with refractory Takayasu arteritis: results from a randomised, double-blind, placebo-controlled, phase 3 trial in Japan (the TAKT study). Ann Rheum Dis. 2018 Mar;77(3):348-54.[Abstract][Full Text]
21. 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 May 15;93(10):1788-90.[Abstract][Full Text]
22. Eichhorn J, Sima D, Thiele B, et al. Anti-endothelial cell antibodies in Takayasu arteritis. Circulation. 1996 Nov 15;94(10):2396-401.[Abstract][Full Text]
23. 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 Mar-Apr;8(2):264-8.[Abstract]
24. Mwipatayi BP, Jeffery PC, Beningfield SJ, et al. Takayasu arteritis: clinical features and management: report of 272 cases. ANZ J Surg. 2005 Mar;75(3):110-7.[Abstract]
25. American Society for Clinical Pathology. Thirty five things physicians and patients should question. Choosing Wisely, an initiative of the ABIM Foundation. 2021 [internet publication].[Full Text]
26. Isselbacher EM, Preventza O, et al. 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022 Dec 13;80(24):e223-393.[Abstract]
27. Treglia GM. Usefulness of whole-body fluorine-18-fluorodeoxyglucose positron emission tomography in patients with large-vessel vasculitis: a systematic review. Clin Rheumatol. 2011 Oct;30(10):1265-75.[Abstract]
28. Webb M, Chambers A, Al-Nahhas A, et al. The role of 18F-FDG PET in characterising disease activity in Takayasu arteritis. Eur J Nucl Med Mol Imaging. 2004 May;31(5):627-34.[Abstract]
29. Kissin EY, Merkel PA. Diagnostic imaging in Takayasu arteritis. Curr Opin Rheumatol. 2004 Jan;16(1):31-7.[Abstract]
30. Arend WP, Michel BA, Block DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990 Aug;33(8):1129-34.[Abstract][Full Text]
31. Yamada I, Nakagawa T, Himeno Y, et al. Takayasu arteritis: evaluation of the thoracic aorta with CT angiography. Radiology. 1998 Oct;209(1):103-9.[Abstract]
32. Aluquin VP, Albano SA, Chan F, et al. Magnetic resonance imaging in the diagnosis and follow up of Takayasu's arteritis in children. Ann Rheum Dis. 2002 Jun;61(6):526-9.[Abstract][Full Text]
33. Andrews J, Al-Nahhas A, Pennell DJ, et al. Non-invasive imaging in the diagnosis and management of Takayasu's arteritis. Ann Rheum Dis. 2004 Aug;63(8):995-1000.[Abstract][Full Text]
34. Lariviere D, Benali K, Coustet B, et al. Positron emission tomography and computed tomography angiography for the diagnosis of giant cell arteritis: A real-life prospective study. Medicine (Baltimore). 2016 Jul;95(30):e4146.[Abstract][Full Text]
35. Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis. Ann Rheum Dis. 2022 Dec;81(12):1654-60.[Abstract][Full Text]
36. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994 Jun 1;120(11):919-29.[Abstract]
37. Hoffman GS, Leavitt RY, Kerr GS, et al. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum. 1994 Apr;37(4):578-82.[Abstract]
38. Hellmich B, Agueda A, Monti S, et al. 2018 update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30.[Abstract][Full Text]
39. Shelhamer JH, Volkman DJ, Parrillo JE, et al. Takayasu's arteritis and its therapy. Ann Intern Med. 1985 Jul;103(1):121-6.[Abstract]
40. Hoffman GS, Merkel PA, Brasington RD, et al. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum. 2004 Jul;50(7):2296-304.[Abstract][Full Text]
41. Molloy ES, Langford CA, Clark CE, et al. Anti-tumor necrosis factor therapy in patients with refractory Takayasu arteritis: long-term follow-up. Ann Rheum Dis. 2008 Nov;67(11):1567-9.[Abstract]
42. Schmidt J, Kermani TA, Bacani AK, et al. Tumor necrosis factor inhibitors in patients with Takayasu arteritis: Experience from a referral center with long-term follow-up. Arthritis Care Res (Hoboken). 2012 Jul;64(7):1079-83.[Abstract]
43. Koster MJ, Matteson EL, Warrington KJ. Recent advances in the clinical management of giant cell arteritis and Takayasu arteritis. Curr Opin Rheumatol. 2016 May;28(3):211-7.[Abstract]
44. Rao SA, Mandalam KR, Rao VR, et al. Takayasu arteritis: initial and long-term follow-up in 16 patients after percutaneous transluminal angioplasty of the descending thoracic and abdominal aorta. Radiology. 1993 Oct;189(1):173-9.[Abstract]
45. Fields CE, Bower TC, Cooper LT, et al. Takayasu's arteritis: operative results and influence of disease activity. J Vasc Surg. 2006 Jan;43(1):64-71.[Abstract][Full Text]
46. Labarca C, Makol A, Crowson CS, et al. Retrospective comparison of open versus endovascular procedures for takayasu arteritis. J Rheumatol. 2016 Feb;43(2):427-32.[Abstract]
47. Miyata T, Sato O, Koyama H, et al. Long-term survival after surgical treatment of patients with Takayasu's arteritis. Circulation. 2003 Sep 23;108(12):1474-80.[Abstract][Full Text]
48. Cui X, Dai X, Ma L, et al. Efficacy and safety of leflunomide treatment in Takayasu arteritis: case series from the East China cohort. Semin Arthritis Rheum. 2020 Feb;50(1):59-65.[Abstract][Full Text]
49. Regola F, Uzzo M, Toniati P, et al. Novel therapies in Takayasu arteritis. Front Med (Lausanne). 2021;8:814075.[Abstract][Full Text]
50. Watanabe R, Hashimoto M. Perspectives of JAK inhibitors for large vessel vasculitis. Front Immunol. 2022;13:881705.[Abstract][Full Text]
51. Bursi R, Cafaro G, Perricone C, et al. Contribution of Janus-kinase/signal transduction activator of transcription pathway in the pathogenesis of vasculitis: a possible treatment target in the upcoming future. Front Pharmacol. 2021;12:635663.[Abstract][Full Text]
52. ClinicalTrails.gov. Efficiency of methotrexate and tofacitinib in mild and moderate patients. Jun 2020 [internet publication].[Full Text]
53. ClinicalTrails.gov. A study to evaluate the efficacy and safety of upadacitinib in participants with Takayasu arteritis (TAK) (SELECT-TAK). May 2023 [internet publication].[Full Text]
54. Langford CA, Cuthbertson D, Ytterberg SR, et al; Vasculitis Clinical Research Consortium. A randomized, double-blind trial of abatacept (CTLA-4Ig) for the treatment of Takayasu arteritis. Arthritis Rheumatol. 2017 Apr;69(4):846-53.[Abstract]
55. Tian X, Li M, Jiang N, et al. Comparative efficacy of secukinumab versus tumor necrosis factor inhibitors for the treatment of Takayasu arteritis. Arthritis Rheumatol. 2023 Aug;75(8):1415-23.[Abstract][Full Text]
56. Maksimowicz-McKinnon K, Hoffman GS. Takayasu arteritis: what is the long-term prognosis? Rheum Dis Clin North Am. 2007 Nov;33(4):777-86.[Abstract]
57. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008 Sep-Oct;26(5 Suppl 51):S94-104.[Abstract]
58. Vanoli M, Daina E, Salvarani C, et al; Itaka Study Group. Takayasu's arteritis: a study of 104 Italian patients. Arthritis Rheum. 2005 Feb 15;53(1):100-7.[Abstract][Full Text]
