Takayasu Arteritis: A current perspective on diagnosis and emerging treatments
DOI:
https://doi.org/10.12775/QS.2025.43.61386Keywords
pulseless disease, Takayasu-Arteritis, vasculitisAbstract
Takayasu-Arteritis (TA), also known as the pulseless disease or aortic arch syndrome is a rare but serious condition that primarily affects mainly the large blood vessels. This inflammatory vascular disease is characterised by intimal fibrosis and vessel narrowing, especially affecting the aorta, its branches and the pulmonary arteries. Patients typically present asymptomatic but such conditions as weak or absent pulses in arms or legs, angina pectoris and stroke-like symptoms can appear in severe cases.
A comprehensive understanding of TA disease is essential for prompt diagnosis and effective management. This review will examine the historical context, symptoms, diagnostic criteria, new therapeutic approaches, and future directions in research and treatment.
References
Arita Y, Ishibashi T, Nakaoka Y. Current immunosuppressive treatment for Takayasu arteritis. Circ J. 2024;88(10):1605-1609. doi:10.1253/circj.CJ-23-0780
Arend W, Michel B, Bloch D, Hunder G. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33(8):1129-1134. doi:10.1002/art.1780330811
Arnaud L, Haroche J, Malek Z, Amoura Z, Mathian A, Gorochov G, et al. Pathogenesis of Takayasu's arteritis: a 2011 update. Autoimmun Rev. 2011;11(1):61-67. doi:10.1016/j.autrev.2011.08.001
Bhandari S, Butt SRR, Ishfaq A, Attaallah MH, Ekhator C, Halappa Nagaraj R, et al. Pathophysiology, diagnosis, and management of Takayasu arteritis: a review of current advances. Cureus. 2023;15(7):e42667. doi:10.7759/cureus.42667
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(Suppl 52):S59-S64.
Chandhu AS, Danda D. Current diagnosis and management of Takayasu arteritis. Int Heart J. 2023;64(4):519-534. doi:10.1536/ihj.23-195. doi: 10.1536/ihj.23-195
El-Reshaid K, Varro J, Al-Attia H, Hegab A, Al-Saleh Q, Al-Mohanadi M, et al. Takayasu's arteritis in Kuwait. J Trop Med Hyg. 1995;98(5):299-305.
Gon Y, Yoshifuji H, Nakajima T, Murakami K, Nakashima R, Ohmura K, et al. Long-term outcomes of refractory Takayasu arteritis patients treated with biologics including ustekinumab. Mod Rheumatol. 2021;31(4):678-683. doi:10.1080/14397595.2020.1800560
Grayson PC, Ponte C, Suppiah R, Robson JC, Gribbons KB, Judge A, Craven A, Khalid S, Hutchings A, Danda D, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group. 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis. Ann Rheum Dis. 2022 Dec;81(12):1654-1660. doi: 10.1136/ard-2022-223482
Gudbrandsson B, Molberg Ø, Garen T, Palm Ø. Prevalence, incidence and disease characteristics of Takayasu arteritis differ by ethnic background: data from a large, population-based cohort in Southern Norway. Arthritis Care Res. 2017;69(2):278-285. doi: 10.1002/acr.22931
Jung JH, Lee YH, Song GG, Jeong HS, Kim JH, Choi SJ. Endovascular versus open surgical intervention in patients with Takayasu's arteritis: a meta-analysis. Eur J Vasc Endovasc Surg. 2018;55(6):888-899. doi:10.1016/j.ejvs.2018.02.030
Kawahito Y, Morinobu A, Kaneko Y, Kohno M, Hirata S, Kishimoto M, et al. Drug treatment algorithm and recommendations from the 2020 update of the Japan College of Rheumatology clinical practice guidelines for the management of rheumatoid arthritis. Mod Rheumatol. 2023;33:21-35. doi:10.1093/mr/roac017
Keser G, Aksu K, Direskeneli H. Takayasu arteritis: an update. Turk J Med Sci. 2018;48(4):681-697. doi:10.3906/sag-1804-136
Kong X, Sun Y, Dai X, Wang L, Ji Z, Chen H, et al. Treatment efficacy and safety of tofacitinib versus methotrexate in Takayasu arteritis: a prospective observational study. Ann Rheum Dis. 2022;81(1):117-123. doi:10.1136/annrheumdis-2021-220832
Li G, Zhao J, Ma Y, Wu W, Jin D, Chen X, et al. Effects of resistance exercise on treatment outcome and laboratory parameters of Takayasu arteritis: a randomized controlled clinical trial. Clin Cardiol. 2020;43(8):864-871. doi:10.1002/clc.23439
Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis. Arthritis Rheumatol. 2021;73(8):1349-1365. doi:10.1002/art.41774
Misra DP, Rathore U, Patro P, Agarwal V, Negi VS. Disease-modifying anti-rheumatic drugs for the management of Takayasu arteritis: a systematic review and meta-analysis. Clin Rheumatol. 2021;40(11):4391-4416. doi:10.1007/s10067-021-05743-2
Mohammad AJ, Mandl T. Takayasu arteritis in southern Sweden. J Rheumatol. 2015;42(5):853-858. doi: 10.3899/jrheum.140843
Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestations of Takayasu arteritis in India and Japan: new classification of angiographic findings. Angiology. 1997;48(5):369-379. doi: 10.1177/000331979704800501
Mutoh T, Ishii T, Shirai T, Akita K, Kamogawa Y, Fujita Y, et al. Refractory Takayasu arteritis successfully treated with rituximab: case-based review. Rheumatol Int. 2019;39(12):1989-1994. doi:10.1007/s00296-019-04390-w
Nakajima M. A case of retinal-venous anastomosis with congenital abnormalities. Nippon Ganka Gakkai Zasshi. 1921;25:399-405.
Nakaoka Y, Isobe M, Takei S, Tanaka Y, Ishii T, Yokota S, et al. Efficacy and safety of tocilizumab in patients with refractory Takayasu arteritis: results from a phase 3 trial in Japan (the TAKT study). Ann Rheum Dis. 2018;77(3):348-354. doi:10.1136/annrheumdis-2017-211878
Nishino Y, Tamai M, Kawakami A, Koga T, Makiyama J, Maeda Y, et al. Serum levels of BAFF for assessing the disease activity of Takayasu arteritis. Clin Exp Rheumatol. 2010;28(1):14-17.
Numano F, Okawara M, Inomata H, Kobayashi Y. Takayasu's arteritis. Lancet. 2000;356(9234):1023-1025. doi:10.1016/S0140-6736(00)02701-X
Onen F, Akkoc N. Epidemiology of Takayasu arteritis. Presse Med. 2017;46(7-8 Pt 2):e197-e203. doi:10.1016/j.lpm.2017.05.034
Park MC, Lee SW, Park YB, Lee SK. Serum cytokine profiles and their correlations with disease activity in Takayasu’s arteritis. Rheumatology. 2006;45(5):545-548. doi: 10.1093/rheumatology/kei266
Park SJ, Kim H, Park YB, Lee SK. Incidence, prevalence, mortality and causes of death in Takayasu arteritis in Korea: a nationwide study. Int J Cardiol. 2017;235:100-104. doi: 10.1016/j.ijcard.2017.02.086
Régnier P, Le Joncour A, Maciejewski-Duval A, Desbois AC, Comarmond C, Rosenzwajg M, et al. Targeting JAK/STAT pathway in Takayasu’s arteritis. Ann Rheum Dis. 2020;79(7):951-959. doi:10.1136/annrheumdis-2019-216900
Renauer P, Sawalha AH. The genetics of Takayasu arteritis. Presse Med. 2017;46(7-8 Pt 2):e179-e187. doi:10.1016/j.lpm.2016.11.031
Renauer PA, Saruhan-Direskeneli G, Coit P, Adler A, Aksu K, Keser G, et al. Identification of susceptibility loci in IL6, RPS9/LILRB3, and an intergenic locus on chromosome 21q22 in Takayasu arteritis in a genome-wide association study. Arthritis Rheumatol. 2015;67(1): 136–143. doi:10.1002/art.39035
Rizzi R, Bruno S. Takayasu's arteritis: a cell-mediated large-vessel vasculitis. Int J Clin Lab Res. 1999;29(1):8-13. doi:10.1007/s005990050055
Rutter M, Bowley J, Lanyon PC, Grainge MJ, Pearce FA. A systematic review and meta-analysis of the incidence rate of Takayasu arteritis. Rheumatology. 2021;60(10):4982-4990
Sanchez-Alvarez C, Crowson CS, Koster MJ, Warrington KJ. Prevalence of Takayasu arteritis: a population-based study. J Rheumatol. 2021;48(6):952-955.doi: 10.3899/jrheum.201463
Sawalha AH, Dozmorov MG. Epigenomic functional characterization of genetic susceptibility variants in systemic vasculitis. J Autoimmun. 2016;67:76–81. doi: 10.1016/j.jaut.2015.10.002
Shao N, Jia H, Li Y, Xu X, Zhang Q. Curcumin improves treatment outcome of Takayasu arteritis patients by reducing TNF-α: a randomized placebo-controlled trial. Immunol Res. 2017;65(5):969-974. doi:10.1007/s12026-017-8917-z
Shimizu K, Sano K. Pulseless disease. Rhinsho Geka. 1948;3:377-396.
Soto ME, Espinola N, Flores-Suarez LF, Reyes PA. Takayasu arteritis: clinical features in 110 Mexican Mestizo patients and cardiovascular impact. Clin Exp Rheumatol. 2008;26(Suppl 49):S9-S15.
Sugiyama K, Tsukahara R, Murayama S. Takayasu disease on the centenary of its discovery. Jpn J Ophthalmol. 2009;53(2):81-91. doi:10.1007/s10384-009-0650-2
Takayasu M. A case with curious change in the central retinal vessel. J Juzen Med Soc. 1908;50:1-6.
Terao C. Revisited HLA and non-HLA genetics of Takayasu arteritis: where are we? J Hum Genet. 2016;61(1):27-32. doi: doi: 10.1038/jhg.2015.87
Terao C, Yoshifuji H, Ohmura K, Matsumura T, Nishio J, Mimori T, et al. Genetic Determinants and an Epistatic Interaction of IL6 and RPS9/LILRB3 Loci in Takayasu Arteritis. Am J Hum Genet. 2013;93(2):289-297. doi:10.1016/j.ajhg.2013.06.010
Vanoli M, Miani S, Amft N, Bacchiani G, Radelli L, Scorza R. Takayasu's arteritis in Italian patients. Clin Exp Rheumatol. 1995;13(1):45-48.
Wang H, Ma J, Wu Q, Luo X, Chen Z, Kou L. Circulating B lymphocytes producing autoantibodies to endothelial cells play a role in the pathogenesis of Takayasu arteritis. J Vasc Surg. 2010;53(1):174-180. doi: 10.1016/j.jvs.2010.06.173
Weyand CM, Goronzy JJ. Molecular approaches toward pathologic mechanisms in giant cell arteritis and Takayasu's arteritis. Curr Opin Rheumatol. 1995;7(1):30-35.
William P., Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33(8):1129-1134. doi:10.1002/art.1780330811. doi: 10.1002/art.1780330803
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Copyright (c) 2025 Nicole Nitschke, Martyna Mocarska, Anna Orłowska, Katarzyna Strakowska, Laura Opalska, Anna Maryńczak, Adrianna Muciek, Jan Mencel, Marta Lenar, Filip Lenar
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