Abstract
Takayasu arteritis (TA) is 1 of the 2 main causes of large vessel vasculitides (LVV), giant cell arteritis being the other. LVV can also develop in various other systemic diseases. In TA, a wide variety of symptoms result from vascular stenoses, occlusions, and dilation. Aneurysms may develop and may occasionally dissect or rupture. Disease activity can sometimes be difficult to assess clinically. Diagnostic modalities also have their shortcomings. Often, acute phase reactants do not accurately detect disease activity. Available vascular imaging modalities may be acceptable in defining vascular anatomy, but are notoriously inaccurate in delineating vascular inflammation. Glucocorticoids remain the cornerstone of therapy in TA, in spite of foreseeable long term side effects. In addition, several steroid-sparing agents are also being used, often based on promising results from small uncontrolled studies. Rarely, endovascular revascularization procedures are necessary. Resection of critical-sized aortic aneurysms and repair of aortic dissections are occasionally warranted as lifesaving procedures. The long term outcome of surgical intervention is often unfavorable and relapses are not uncommon. In addition to TA, other less commonly encountered causes of LVV are also briefly discussed in this review.
Similar content being viewed by others
References
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Takayasu arteritis and giant cell arteritis: a spectrum within the same disease? Medicine. 2009;88:221–6. This retrospective review led to the introduction of the hypothesis that TA and GCA may represent different phenotypes within the spectrum of a single disorder.
Tso E, Flamm SD, White RD, Schvartzman PR, Mascha E, Hoffman GS. Takayasu arteritis: utility and limitations of magnetic resonance imaging in diagnosis and treatment. Arthritis Rheum. 2002;46:1634–42.
Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: new classification. Int J Cardiol. 1996;54(Suppl):S155–63.
Lupi-Herrera E, Sanchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE. Takayasu's arteritis. Clinical study of 107 cases. Am Heart J. 1977;93:94–103.
Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33:1129–34.
Dabague J, Reyes PA. Takayasu arteritis in Mexico: a 38-year clinical perspective through literature review. Int J Cardiol. 1996;54(Suppl):S103–9.
Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder GG. Takayasu arteritis. A study of 32 North American patients. Medicine. 1985;64:89–99.
Ishikawa K. Natural history and classification of occlusive thromboaortopathy (Takayasu’s disease). Circulation. 1978;57:27–35.
Koide K. Takayasu arteritis in Japan. Heart Vessels. 1992;7(Suppl):48–54.
Kimura A, Kitamura H, Date Y, Numano F. Comprehensive analysis of HLA genes in Takayasu arteritis in Japan. Int J Cardiol. 1996;54(Suppl):S61–9.
Cid MC, Font C, Coll-Vinent B, Grau JM. Large vessel vasculitides. Curr Opin Rheumatol. 1998;10:18–28.
Lupi E, Sanchez G, Horwitz S, Gutierrez E. Pulmonary artery involvement in Takayasu’s arteritis. Chest. 1975;67:69–74.
Sharma BK, Jain S, Sagar S. Systemic manifestations of Takayasu arteritis: the expanding spectrum. Int J Cardiol. 1996;54(Suppl):S149–54.
Grayson PC, Tomasson G, Cuthbertson D, Carette S, Hoffman GS, Khalidi NA, et al. Association of vascular physical examination findings and arteriographic lesions in large vessel vasculitis. J Rheumatol. 2012;39:303–9.
Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, et al. Takayasu arteritis. Ann Intern Med. 1994;120:919–29.
Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000–9.
Kermani TA, Warrington KJ. Recent advances in diagnostic strategies for giant cell arteritis. Curr Neurol Neurosci Rep. 2012;12:138–44.
Blockmans D, Bley T, Schmidt W. Imaging for large-vessel vasculitis. Curr Opin Rheumatol. 2009;21:19–28.
Aydin SZ, Yilmaz N, Akar S, Aksu K, Kamali S, Yucel E, et al. Assessment of disease activity and progression in Takayasu’s arteritis with Disease Extent Index-Takayasu. Rheumatology. 2010;49:1889–93.
Misra R, Danda D, Rajappa SM, Ghosh A, Gupta R, Mahendranath KM, et al. Development and initial validation of the Indian Takayasu Clinical Activity Score (ITAS2010). Rheumatology. 2013;52:1795–801.
Lagneau P, Michel JB, Vuong PN. Surgical treatment of Takayasu’s disease. Ann Surg. 1987;205:157–66.
Clifford A, Clark T, Johnston D, Petterson G, Roselli E, Rodriguez E, et al. Large Vessel vasculitis: estimating disease activity in patients with inflammatory thoracic aortic aneurysms. Arthritis Rheum. 2013;65:S10. Abstract.
Wong VC, Wang RY, Tse TF. Pregnancy and Takayasu’s arteritis. Am J Med. 1983;75:597–601.
Bicakcigil M, Aksu K, Kamali S, Ozbalkan Z, Ates A, Karadag O, et al. Takayasu's arteritis in Turkey—clinical and angiographic features of 248 patients. Clin Exp Rheumatol. 2009;27(1 Suppl 52):S59–64.
Schmidt J, Kermani TA, Bacani AK, Crowson CS, Cooper LT, Matteson EL, et al. Diagnostic features, treatment, and outcomes of Takayasu arteritis in a US cohort of 126 patients. Mayo Clin Proc. 2013;88:822–30. Report of a large cohort of patients with TA enrolled in a single center over 25 years.
Hoffman GS, Leavitt RY, Kerr GS, Rottem M, Sneller MC, Fauci AS. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum. 1994;37:578–82.
Valsakumar AK, Valappil UC, Jorapur V, Garg N, Nityanand S, Sinha N. Role of immunosuppressive therapy on clinical, immunological, and angiographic outcome in active Takayasu’s arteritis. J Rheumatol. 2003;30:1793–8.
Shinjo SK, Pereira RM, Tizziani VA, Radu AS, Levy-Neto M. Mycophenolate mofetil reduces disease activity and steroid dosage in Takayasu arteritis. Clin Rheumatol. 2007;26:1871–5.
Goel R, Danda D, Mathew J, Edwin N. Mycophenolate mofetil in Takayasu’s arteritis. Clin Rheumatol. 2010;29:329–32.
de Souza AW, da Silva MD, Machado LS, Oliveira AC, Pinheiro FA, Sato EI. Short-term effect of leflunomide in patients with Takayasu arteritis: an observational study. Scand J Rheumatol. 2012;41:227–30.
de Souza AW, Machado NP, Pereira VM, Arraes AE, Reis Neto ET, Mariz HA, et al. Antiplatelet therapy for the prevention of arterial ischemic events in Takayasu arteritis. Circ J. 2010;74:1236–41.
Hoffman GS, Merkel PA, Brasington RD, Lenschow DJ, Liang P. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum. 2004;50:2296–304.
Molloy ES, Langford CA, Clark TM, Gota CE, Hoffman GS. Anti-tumour necrosis factor therapy in patients with refractory Takayasu arteritis: long-term follow-up. Ann Rheum Dis. 2008;67:1567–9.
Schmidt J, Kermani TA, Bacani AK, Crowson CS, Matteson EL, Warrington KJ. Tumor necrosis factor inhibitors in patients with Takayasu arteritis: experience from a referral center with long-term follow-up. Arthritis Care Res. 2012;64:1079–83. Largest published series of TA patients demonstrating efficacy of treatment with TNF inhibitors.
Mekinian A, Neel A, Sibilia J, Cohen P, Connault J, Lambert M, et al. Efficacy and tolerance of infliximab in refractory Takayasu arteritis: French multi-centre study. Rheumatology. 2012;51:882–6.
Quartuccio L, Schiavon F, Zuliani F, Carraro V, Catarsi E, Tavoni AG, et al. Long-term efficacy and improvement of health-related quality of life in patients with Takayasu’s arteritis treated with infliximab. Clin Exp Rheumatol. 2012;30:922–8.
Unizony S, Arias-Urdaneta L, Miloslavsky E, Arvikar S, Khosroshahi A, Keroack B, et al. Tocilizumab for the treatment of large-vessel vasculitis (giant cell arteritis, Takayasu arteritis) and polymyalgia rheumatica. Arthritis Care Res. 2012;64:1720–9. Largest published case series of tocilizumab therapy in large-vessel vasculitis.
Abisror N, Mekinian A, Lavigne C, Vandenhende MA, Soussan M, Fain O. Tocilizumab in refractory Takayasu arteritis: a case series and updated literature review. Autoimmun Rev. 2013;12(12):1143–9. Report on a compilation of 44 cases reviewing the efficacy of tocilizumab in refractory Takayasu arteritis.
Clifford A, Hoffman GS. Recent advances in the medical management of Takayasu arteritis: an update on use of biologic therapies. Curr Opin Rheumatol. 2014;26:7–15. A nice review on the use of biologic therapies in TA.
Keser G, Direskeneli H, Aksu K. Management of Takayasu arteritis: a systematic review. Rheumatology. 2013.
Unizony S, Stone JH, Stone JR. New treatment strategies in large-vessel vasculitis. Curr Opin Rheumatol. 2013;25:3–9. A very elegant review of biologic response modifiers in the treatment of large-vessel vasculitis.
Ernst D, Greer M, Stoll M, Meyer-Olson D, Schmidt RE, Witte T. Remission achieved in refractory advanced Takayasu arteritis using rituximab. Case Rep Rheumatol. 2012;2012:406963.
Hoyer BF, Mumtaz IM, Loddenkemper K, Bruns A, Sengler C, Hermann KG, et al. Takayasu arteritis is characterised by disturbances of B cell homeostasis and responds to B cell depletion therapy with rituximab. Ann Rheum Dis. 2012;71:75–9.
Kim YW, Kim DI, Park YJ, Yang SS, Lee GY, Kim DK, et al. Surgical bypass vs endovascular treatment for patients with supra-aortic arterial occlusive disease due to Takayasu arteritis. J Vasc Surg. 2012;55:693–700.
Lee G, Jeon P, Do YS, Sung K, Kim DI, Kim YW, et al. Comparison of outcomes between endovascular treatment and bypass surgery in Takayasu arteritis. Scand J Rheumatol. 2013.
Qureshi MA, Martin Z, Greenberg RK. Endovascular management of patients with Takayasu arteritis: stents versus stent grafts. Semin Vasc Surg. 2011;24:44–52.
Saadoun D, Lambert M, Mirault T, Resche-Rigon M, Koskas F, Cluzel P, et al. Retrospective analysis of surgery versus endovascular intervention in Takayasu arteritis: a multicenter experience. Circulation. 2012;125:813–9.
Maksimowicz-McKinnon K, Hoffman GS. Takayasu arteritis: what is the long-term prognosis? Rheum Dis Clin N Am. 2007;33:777–86. vi.
Koc Y, Gullu I, Akpek G, Akpolat T, Kansu E, Kiraz S, et al. Vascular involvement in Behcet’s disease. J Rheumatol. 1992;19:402–10.
Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behcet disease. Curr Opin Rheumatol. 2005;17:1–8.
Uzun O, Akpolat T, Erkan L. Pulmonary vasculitis Behcet disease: a cumulative analysis. Chest. 2005;127:2243–53.
Seyahi E, Melikoglu M, Akman C, Hamuryudan V, Ozer H, Hatemi G, et al. Pulmonary artery involvement and associated lung disease in Behcet disease: a series of 47 patients. Medicine. 2012;91:35–48.
Gravallese EM, Corson JM, Coblyn JS, Pinkus GS, Weinblatt ME. Rheumatoid aortitis: a rarely recognized but clinically significant entity. Medicine. 1989;68:95–106.
Kawasuji M, Hetzer R, Oelert H, Stauch G, Borst HG. Aortic valve replacement and ascending aorta replacement in ankylosing spondylitis: report of three surgical cases and review of the literature. Thorac Cardiovasc Surg. 1982;30:310–4.
Stamp L, Lambie N, O'Donnell J. HLA-B27 associated spondyloarthropathy and severe ascending aortitis. J Rheumatol. 2000;27:2038–40.
Haynes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine. 1980;59:426–41.
Selim AG, Fulford LG, Mohiaddin RH, Sheppard MN. Active aortitis in relapsing polychondritis. J Clin Pathol. 2001;54:890–2.
Rho YH, Choi SJ, Choi YS, Lee YH, Ji JD, Song GG. Relapsing polychondritis with aortitis without valvular involvement. J Rheumatol. 2005;32:954–6.
Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23:88–94. A comprehensive review on IgG4-related systemic disease as a cause of thoracic aortitis, inflammatory abdominal aortic aneurysm or peri-aortitis, and retroperitoneal fibrosis.
Compliance with Ethics Guidelines
Conflict of Interest
Soumya Chatterjee and Carmela D. Tan declare that they have no conflict of interest. Scott D. Flamm has been a consultant for Bayer Healthcare on the Cardiac Imaging Advisory Board. E. Rene Rodriguez declares that he has no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Author information
Authors and Affiliations
Corresponding author
Additional information
This article is part of the Topical Collection on Peripheral Vascular Disease
Rights and permissions
About this article
Cite this article
Chatterjee, S., Flamm, S.D., Tan, C.D. et al. Clinical Diagnosis and Management of Large Vessel Vasculitis: Takayasu Arteritis. Curr Cardiol Rep 16, 499 (2014). https://doi.org/10.1007/s11886-014-0499-y
Published:
DOI: https://doi.org/10.1007/s11886-014-0499-y
Keywords
- Large vessel vasculitis
- Takayasu arteritis
- Claudication
- Stenosis
- Occlusion
- Ectasia
- Aneurysm
- Dissection
- Acute phase reactant
- ESR
- CRP
- Interleukin-6
- Computerized tomographic angiogram (CTA)
- Magnetic resonance angiogram (MRA)
- Fluorodeoxyglucose(18F)-positron emission tomography (FDG-PET)
- Vascular Doppler ultrasound
- Glucocorticoid
- Prednisone
- Methylprednisolone
- Methotrexate
- Azathioprine
- Leflunomide
- Cyclophosphamide
- Tocilizumab
- Rituximab
- TNF inhibitors
- Angioplasty
- Stent
- Bypass