Opinion statement
Takayasu arteritis is a large vessel vasculitis of unknown aetiology, more common in the tropics. Assessment of disease activity is challenging and evolving, with limitations in the use of conventional inflammatory markers like ESR and CRP resulting in utilization of composite clinical (Kerr criteria, ITAS2010) and imaging (angiography and FDG-PET) modalities. Management is challenging, with a paucity of high-quality evidence to guide therapy. Conventional disease-modifying agents are commonly used, although evidence base is limited, with methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide and leflunomide showing efficacy in open label studies. Role of biologic agents like anti-TNF-alpha agents, tocilizumab and rituximab is based on open label evidence, with a recent randomized trial failing to show significant efficacy of abatacept in reducing disease relapses. Endovascular stenting and open surgical revascularisation, generally done when disease is inactive, help in restoration of blood flow supplied by stenosed segments; however, these may require to be repeated as restenosis is not rare.
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Takayasu M. A case with peculiar changes of the central retinal vessels. Acta Soc Ophthalmol Jap. 1908;12:554.
Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33:1129–34.
• Serra R, Butrico L, Fugetto F, et al. Updates in pathophysiology, diagnosis and management of Takayasu arteritis. Ann Vasc Surg. 2016;35:210–25. This is a comprehensive review of latest data and evidences in epidemiology, pathogenesis, clinical manifestations, diagnosis, assessment of disease activity and treatment of Takayasu arteritis
Chauhan SK, Tripathy NK, Nityanand S. Antigenic targets and pathogenicity of anti–aortic endothelial cell antibodies in Takayasu arteritis. Arthritis Rheum. 2006;54:2326–33.
Tripathy NK, Sinha N, Nityanand S. Anti-annexin V antibodies in Takayasu's arteritis: prevalence and relationship with disease activity. Clin Exp Immunol. 2003;134:360–4.
Hoffman GS. Takayasu arteritis: lessons from the American National Institutes of Health Experience. Int J Cardiol. 1996;54:S99–10.
Cong XL, Dai SM, Feng X, et al. Takayasu's arteritis: clinical features and outcomes of 125 patients in China. Clin Rheumatol. 2010;29:973–81.
Sharma BK, Jain S, Radotra BD. An autopsy study of Takayasu arteritis in India. Int J Cardiol. 1998;66(Suppl 1):S85–90.
Tamura N, Maejima Y, Tezuka D, et al. Profiles of serum cytokine levels in Takayasu arteritis patients: potential utility as biomarkers for monitoring disease activity. J Cardiol. 2016; doi:10.1016/j.jjcc.2016.10.016.
Goel R, Kabeerdoss J, Ram B, et al. Serum cytokine profile in Asian Indian patients with Takayasu arteritis and its association with disease activity. Open Rheumatol J. 2017;11:23–9.
Dagna L, Salvo F, Tiraboschi M, et al. Pentraxin-3 as a marker of disease activity in Takayasu arteritis. Ann Intern Med. 2011;155:425–33.
Ishihara T, Haraguchi G, Tezuka D, et al. Diagnosis and assessment of Takayasu arteritis by multiple biomarkers. Circ J. 2013;77:477–83.
Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994;120:919–29.
Aydin SZ, Yilmaz N, Akar S, et al. Assessment of disease activity and progression in Takayasu’s arteritis with disease extent index-Takayasu. Rheumatology. 2010;49:1889–93.
Sivakumar MR, Mishra RN, Bacon PA. The Indian perspective of Takayasu arteritis and development of a disease extent index (DEI.Tak) to assess Takayasu arteritis. Rheumatology. 2005;44:iii6–7.
Misra R, Danda D, Rajappa SM, Indian Rheumatology Vasculitis (IRAVAS) Group, et al. Development and initial validation of the Indian Takayasu clinical activity score (ITAS2010). Rheumatology. 2013;52:1795–801.
• Alibaz-Oner F, Aydin SZ, Direskeneli H. Recent advances in Takayasu’s arteritis. Eur J Rheumatol. 2015;2:24–30. This review summarises the recent developments in the diagnosis, clinical course, disease assessment with biomarkers/imaging and new clinical tools, patient-reported outcomes and new treatment options in Takayasu arteritis
Sinha D, Mondal S, Nag A, et al. Development of a colour Doppler ultrasound scoring system in patients of Takayasu’s arteritis and its correlation with clinical activity score (ITAS 2010). Rheumatology. 2013;52:2196–202.
Cheng Y, Lv N, Wang Z, et al. 18-FDG-PET in assessing disease activity in Takayasu arteritis: a meta-analysis. Clin Exp Rheumatol. 2013;31:S22–7.
Santhosh S, Mittal BR, Gayana S, et al. F-18 FDG PET/CT in the evaluation of Takayasu arteritis: an experience from the tropics. J Nucl Cardiol. 2014;21:993–1000.
Incerti E, Tombetti E, Fallanca F, et al. 18F-FDG PET reveals unique features of large vessel inflammation in patients with Takayasu's arteritis. Eur J Nucl Med Mol Imaging. 2017;44:1109–18.
Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2009;68:318–23.
•• Ferfar Y, Mirault T, Desbois AC, et al. Biotherapies in large vessel vasculitis. Autoimmun Rev. 2016;15:544–51. This is a systematic review of biological therapies in large vessel vasculitis
Hunder GG, Sheps SG, Allen GL, et al. Daily and alternate-day corticosteroid regimens in treatment of giant cell arteritis: comparison in a prospective study. Ann Intern Med. 1975;82:613–8.
Hoffman GS, Leavitt RY, Kerr GS, et al. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum. 1994;37:578–82.
Gokhale Y, Bedmutha K, Kamble A, et al. P74. Response of Takayasu arteritis (TA) to prednisolone and methotrexate: an open label study [abstract]. Indian J Rheumatol. 2013;8:S27.
Valsakumar AK, Valappil UC, Jorapur V, et al. Role of immunosuppressive therapy on clinical, immunological, and angiographic outcome in active Takayasu's arteritis. J Rheumatol. 2003;30:1793–8.
Daina E, Schieppati A, Remuzzi G. Mycophenolate mofetil for the treatment of Takayasu arteritis: report of three cases. Ann Intern Med. 1999;130:422–6.
Shinjo SK, Pereira RMR, Tizziani VAP, et al. Mycophenolate mofetil reduces disease activity and steroid dosage in Takayasu arteritis. Clin Rheumatol. 2007;26:1871–5.
Goel R, Danda D, Mathew J, et al. Mycophenolate mofetil in Takayasu's arteritis. Clin Rheumatol. 2010;29:329–32.
Dai D, Wang Y, Jin H, et al. The efficacy of mycophenolate mofetil in treating Takayasu arteritis: a systematic review and meta-analysis. Rheumatol Int. 2017; doi:10.1007/s00296-017-3704-7. This is a systematic review on the use of mycophenolate mofetil in Takayasu arteritis
de Souza AWS, da Silva MD, MacHado LSG, et al. Short-term effect of leflunomide in patients with Takayasu arteritis: an observational study. Scand J Rheumatol. 2012;41:227–30.
de Souza AWS, de Almeida AR, de Cinque AH, et al. Leflunomide in Takayasu arteritis—a long term observational study. Rev Bras Reumatol Engl Ed. 2016;56:371–5.
Henes JC, Müller M, Pfannenberg C, et al. Cyclophosphamide for large-vessel vasculitis: assessment of response by PET/CT. Clin Exp Rheumatol. 2011;29:S43–8.
Stern S, Clemente G, Reiff A, et al. Treatment of pediatric Takayasu arteritis with infliximab and cyclophosphamide: experience from an American-Brazilian cohort study. J Clin Rheumatol. 2014;20:183–8.
Shimizu M, Ueno K, Ishikawa S, et al. Successful multitarget therapy using mizoribine and tacrolimus for refractory Takayasu arteritis. Rheumatology. 2014;53:1530–2.
Yamazaki H, Nanki T, Harigai M, et al. Successful treatment of refractory Takayasu arteritis with tacrolimus. J Rheumatol. 2012;39:1487–8.
Yokoe I, Haraoka H, Harashima H. A patient with Takayasu’s arteritis and rheumatoid arthritis who responded to tacrolimus hydrate. Intern Med. 2007;46:1873–7.
Agarwal V, Gupta L, Misra R, et al. Is pre-pulseless Takayasu’s arteritis always treatable? Ann Clin Case Rep. 2017;2:1307.
Tripathy NK, Gupta PC, Nityanand S. High TNF-α and low IL-2 producing T cells characterize active disease in Takayasu’s arteritis. Clin Immunol. 2006;118:154–8.
• Osman M, Pagnoux C, Dryden DM, et al. The role of biological agents in the management of large vessel vasculitis (LVV): a systematic review and meta-analysis. PLoS One. 2014;9:e115026. This is a systematic review on the role of biological agents in treatment of large vessel vasculitis
Vinicki JP, Garcia-Vicuna R, Arredondo M, et al. Sustained remission after long-term biological therapy with large vessel vasculitis: an analysis of ten cases. Reumatol Clin. 2016; doi:10.1016/j.reuma.2016.06.003.
Park MC, Lee SW, Park YB, et al. Serum cytokine profiles and their correlations with disease activity in Takayasu’s arteritis. Rheumatology. 2006;45:545–8.
Osman M, Emery D, Yacyshyan E. Tocilizumab for treating Takayasu’s arteritis and associated stroke: a case series and updated review of the literature. J Stroke Cerebrovasc Dis. 2015;24:1291–8.
Batu ED, Sonmez HE, Hazirolan T, et al. Tocilizumab treatment in childhood Takayasu arteritis: case series of four patients and systematic review of the literature. Semin Arthritis Rheum. 2017;46:529–35.
Loricera J, Blanco R, Castaneda S, et al. Tocilizumab in refractory aortitis: study on 16 patients and literature review. Clin Exp Rheumatol. 2014; 32: S79–89.
Goel R, Danda D, Kumar S, et al. Rapid control of disease activity by tocilizumab in 10 ‘difficult-to-treat’ cases of Takayasu arteritis. Int J Rheum Dis. 2013; 16: 754–61.
Langford CA, Cuthbertson D, Ytterberg SR, et al. A randomized, double-blind trial of Abatacept (CTLA4Ig) for the treatment of Takayasu arteritis. Arthritis Rheum. 2017;69:846–53.
Terao C, Yoshifuji H, Kimaru A, et al. Two susceptibility loci to Takayasu arteritis reveal a synergistic role of the IL12B and HLA-B regions in a Japanese population. Am J Hum Genet. 2013;93:289–97.
Saadoun D, Garrido M, Comarmond C, et al. Th1 and th17 cytokines drive inflammation in Takayasu arteritis. Arthritis Rheumatol. 2015;67:1353–60.
Misra DP, Chaurasia S, Misra R. Increased circulating Th17 cells, serum IL-17A, and IL-23 in Takayasu arteritis. Autoimmune Dis. 2016;2016:7841718.
Terao C, Yoshifuji H, Nakajima T, et al. Ustekinumab as a therapeutic option for Takayasu arteritis: from genetic findings to clinical application. Scand J Rheumatol. 2015;27:1–3.
Galarza C, Valencia D, Tobon GJ, et al. Should rituximab be considered as the first-choice treatment for severe autoimmune rheumatic diseases? Clin Rev Allergy Immunol. 2008;34:124–8.
Hoyer BF, Mumtaz IM, Loddenkemper K, 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.
Ernst D, Greer M, Stoll M, et al. Remission achieved in refractory advanced Takayasu arteritis using rituximab. Case Rep Rheumatol. 2012;2012:406963.
Caltran E, Di Colo G, Ghigliotti G, et al. Two Takayasu arteritis patients successfully treated with rituximab. Clin Rheumatol. 2014;33:1183–4.
Shi G, Hua M, Xu Q, et al. Resveratrol improves treatment outcome and laboratory parameters in patients with Takayasu arteritis: a randomized double-blind and placebo-controlled trial. Immunobiology. 2017;222:164–8.
Shao N, Jia H, Li Y, et al. Curcumin improves treatment outcome of Takayasu arteritis patients by reducing TNF-α: a randomized placebo-controlled double-blind clinical trial. Immunol Res. 2017; doi:10.1007/s12026-017-8917-z.
• Misra DP, Sharma A, Kadhiravan T, et al. A scoping review of the use of non-biologic disease modifying anti-rheumatic drugs in the management of large vessel vasculitis. Autoimmun Rev. 2017;16:179–91. This review discusses in-depth the current evidence regarding use of non-biologic disease modifying agents in TA and giant cell arteritis
Fields CE, Bower TC, Cooper LT, et al. Takayasu’s arteritis: operative results and influence of disease activity. J Vasc Surg. 2006;43:64–71.
Perera AH, Youngstein T, Gibbs RG, et al. Optimizing the outcome of vascular intervention for Takayasu arteritis. Br J Surg. 2014;101:43–50.
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.
•• Mason JC. Takayasu arteritis: surgical interventions. Curr Opin Rheumatol. 2015;27:45–52. A comprehensive review on surgical management of Takayasu arteritis
Saadoun D, Lambert M, Mirault T, et al. Retrospective analysis of surgery versus endovascular intervention in Takayasu arteritis: a multicentre experience. Circulation. 2012;125:813–9.
Park HS, Do YS, Park KB, et al. Long-term results of endovascular treatment in renal arterial stenosis from Takayasu arteritis: angioplasty versus stent placement. Eur J Radiol. 2013;82:1913–8.
Weaver FA, Kumar SR, Yellin AE, et al. Renal revascularization in Takayasu arteritis-induced renal artery stenosis. J Vasc Surg. 2004;39:749–57.
Kim YW, Kim DI, Park YJ, et al. Surgical bypass vs endovascular treatment for patients with supra-aortic arterial occlusive disease due to Takayasu arteritis. J Vas Surg. 2012;55:693–700.
Tyagi S, Verma PK, Gambhir DS, et al. Early and long-term results of subclavian angioplasty in aortoarteritis (Takayasu disease): comparison with atherosclerosis. Cardiovasc Intervent Radiol. 1998;21:219–24.
Endo M, Tomizawa Y, Nishida H, et al. Angiographic findings and surgical treatments of coronary artery involvement in Takayasu arteritis. J Thorac Cardiovasc Surg. 2003;125:570–7.
Lee K, Kang WC, Ahn T, et al. Long-term outcome of drug-eluting stent for coronary artery stenosis in Takayasu’s arteritis. Int J Cardiol. 2010;145:532–5.
Kieffer E, Chiche L, Bertal A, et al. Descending thoracic and thoracoabdominal aortic aneurysm in patients with Takayasu’s disease. Ann Vasc Surg. 2004;18:505–13.
Baril DT, Carroccio A, Palchik E, et al. Endovascular treatment of complicated aortic aneurysms in patients with underlying arteriopathies. Ann Vasc Surg. 2006;20:464–71.
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GSRSNK Naidu, Durga Prasanna Misra and Aman Sharma have no conflict of interest.
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Naidu, G., Misra, D.P. & Sharma, A. Current and Future Treatment Options for Takayasu Arteritis and Persistent Therapeutic Challenges. Curr Treat Options in Rheum 3, 141–152 (2017). https://doi.org/10.1007/s40674-017-0070-2
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DOI: https://doi.org/10.1007/s40674-017-0070-2