Takayasu arteritis

Overview

Takayasu arteritis, also known as Takayasu’s arteritis and historically as pulseless disease, aortic arch syndrome, or occlusive thromboaortopathy, is a rare autoimmune large-vessel vasculitis that primarily affects the aorta and its major branches, although the pulmonary arteries may also be involved. The immune system mistakenly attacks the walls of affected arteries, causing granulomatous inflammation that can lead to thickening of the vessel wall, narrowing (stenosis), blockage (occlusion), or, less commonly, aneurysms.

Common Symptoms

Symptoms occur in two phases:

The first, or systemic phase, reflects active inflammation and may include fever, fatigue, weight loss, arthritis, and non-specific aches and pains.

The second, or occlusive phase, results from narrowing or blockage of affected arteries and may include limb pain during activity (claudication), vision problems, dizziness or fainting, headaches, reduced or absent pulses, differences in blood pressure between the arms, and, in some cases, chest pain or high blood pressure depending on which arteries are involved.

Coexisting Diseases and Conditions

Takayasu arteritis may occur alongside other inflammatory and autoimmune diseases, most commonly inflammatory bowel disease (Crohn’s disease and ulcerative colitis), ankylosing spondylitis, Behçet syndrome, and autoimmune thyroid disease. Less commonly, it has also been associated with Sjögren disease, systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, autoimmune hepatitis, and psoriasis.

Risk Factors and Prevalence

Takayasu arteritis is a rare disease that most often develops before age 40 and affects females much more frequently than males. Although it occurs worldwide, it is more common in people of Asian ancestry and is particularly prevalent in East and Southeast Asia. Genetic susceptibility, including certain human leukocyte antigen (HLA) genes, is believed to contribute to disease risk.

Recent Research

Sources

Sources
1. Tombetti, E., & Mason, J. C. (2019). Takayasu arteritis: advanced understanding is leading to new horizons. Rheumatology (Oxford, England), 58(2), 206–219. https://doi.org/10.1093/rheumatology/key040
2. Tacoy G. (2018). Management of Takayasu arteritis. Future cardiology, 14(2), 105–108. https://doi.org/10.2217/fca-2017-0030
3. F Guédon, A., Bourgade, R., Elhannani, M., Toquet, C., Espitia, O., Fain, O., & Mekinian, A. (2023). Artérite de Takayasu [Takayasu’s arteritis]. La Revue du praticien, 73(4), 400–405.
4. Kerr G. (1994). Takayasu’s arteritis. Current opinion in rheumatology, 6(1), 32–38. https://doi.org/10.1097/00002281-199401000-00006
5. Esatoglu, Sinem Nihal; Hatemi, Gulen. Takayasu arteritis. Current Opinion in Rheumatology 34(1):p 18-24, January 2022. | DOI: 10.1097/BOR.0000000000000852
6. Espinoza, J. L., Ai, S., & Matsumura, I. (2018). New Insights on the Pathogenesis of Takayasu Arteritis: Revisiting the Microbial Theory. Pathogens, 7(3), 73. https://doi.org/10.3390/pathogens7030073
7. Takayasu’s Arteritis. (n.d.). Retrieved from https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Takayasus-Arteritis
8. What is the difference between Takayasu’s and giant cell arteritis? (n.d.). Vasculitis Foundation. Retrieved from https://www.vasculitisfoundation.org/mcm_faq/what-is-the-difference-between-takayasus-and-giant-cell-arteritis/
9. Wan, J., et al. (2020). Comparison of Clinical Features at the Onset of Takayasu’s Arteritis According to Age and Sex. Current vascular pharmacology, 18(1), 80–86. https://doi.org/10.2174/1570161117666190621152506
10. Moisii, P., Jari, I., Naum, A. G., Butcovan, D., & Tinica, G. (2024). Takayasu’s Arteritis: A Special Case Report and Review of the Literature. Medicina (Kaunas, Lithuania), 60(3), 456. https://doi.org/10.3390/medicina60030456
11. Takayasu’s arteritis. (1977). British medical journal, 1(6062), 667.
12. Esatoglu, S. N., Ok, A. M., Ucar, D., Celik, A. F., Ugurlu, S., Hamuryudan, V., Yazici, H., & Seyahi, E. (2020). Takayasu’s arteritis: associated inflammatory diseases. Clinical and experimental rheumatology, 38 Suppl 124(2), 61–68.
13. Keser, G., Aksu, K., & Direskeneli, H. (2018). Takayasu arteritis: an update. Turkish journal of medical sciences, 48(4), 681–697. https://doi.org/10.3906/sag-1804-136
14. Kushwah, S., Ramanan, A. V., & Bagri, N. K. (2023). Infantile Takayasu arteritis: how is it different?. Rheumatology (Oxford, England), 62(9), 2946–2947. https://doi.org/10.1093/rheumatology/kead131