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  • Review Article
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Advances in the pathophysiology, diagnosis and treatment of Takayasu arteritis

Nature Reviews Rheumatology (2025)Cite this article

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Abstract

Takayasu arteritis (TAK) is a rare, chronic, large-vessel vasculitis that primarily targets the aorta and its major branches, leading to vascular stenosis, occlusion and aneurysm formation. TAK, which is characterized by granulomatous inflammation of the arterial wall, predominantly affects women, with peak onset typically occurring between 20 and 40 years of age. The disease exhibits substantial geographic variability in prevalence, with emerging evidence suggesting that these differences are partly owing to variations in genetic susceptibility loci, particularly within immune-related genes; however, the role of environmental factors in the disease aetiology remains poorly understood. Non-invasive imaging techniques have become central to both diagnosis and disease monitoring. Furthermore, the development of biomarkers holds promise for more accurate assessment of disease activity. The management of TAK is evolving, driven by an improved understanding of disease pathogenesis. The growing use of biologic agents is providing new treatment options, particularly for patients with refractory or relapsing disease. By integrating these developments, this Review is aimed at serving as a comprehensive resource for clinicians and researchers dedicated to improving the understanding and management of TAK.

Key points

  • Takayasu arteritis (TAK) is a rare large-vessel vasculitis with a global incidence of 1.11 per million person-years, which predominantly affects young women 20–40 years of age.

  • The pathogenesis of TAK involves multiple interconnected immune-mediated processes that ultimately result in vascular fibrosis and stenosis.

  • Over 100 genetic susceptibility loci, including HLA-B*52, have been identified, contributing to geographic differences in disease prevalence.

  • Non-invasive imaging is central to diagnosis and monitoring; 18F-fluorodeoxyglucose-PET uptake in the arterial wall predicts future stenotic lesions better than wall thickness alone.

  • Serum pentraxin-3 shows promise as a biomarker of disease activity, particularly when IL-6 blockers are used.

  • Glucocorticoids combined with conventional immunosuppressive therapy remain the cornerstone of treatment; relapse rates with glucocorticoid monotherapy reach 60–77% within 1 year.

  • Biologic agents, including TNF inhibitors and tocilizumab, can be effective in refractory TAK; therapies such as secukinumab and Janus kinase inhibitors are emerging options.

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Fig. 1: Pathology of Takayasu arteritis.
Fig. 2: Therapeutic targets in Takayasu arteritis.
Fig. 3: Angiographic patterns of Takayasu arteritis.
Fig. 4: Therapeutic approaches to Takayasu arteritis.

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Acknowledgements

The authors dedicate this work to the memory of Dr. Debashish Danda, whose pioneering contributions profoundly shaped our understanding of this complex disease and advanced patient care. A distinguished expert, esteemed colleague, dedicated mentor, passionate educator and cherished friend, his legacy continues to guide and inspire both the vasculitis field and the global rheumatology community. K.A.Q. and P.C.G. are funded by the Intramural Research Program at the National Institute of Arthritis and Musculoskeletal and Skin Diseases at the US National Institutes of Health. The findings and conclusions presented in this paper are those of the authors and do not necessarily reflect the views of the US National Institutes of Health or the US Department of Health and Human Services.

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Authors and Affiliations

  1. Departments of Pediatrics, Medicine, and Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    Amr H. Sawalha

  2. Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

    Durga P. Misra

  3. Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India

    Ruchika Goel

  4. Department of Internal Medicine, Division of Rheumatology, Marmara University Faculty of Medicine, Istanbul, Turkey

    Fatma Alibaz-Oner & Haner Direskeneli

  5. National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, USA

    Kaitlin A. Quinn & Peter C. Grayson

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Sawalha, A.H., Misra, D.P., Goel, R. et al. Advances in the pathophysiology, diagnosis and treatment of Takayasu arteritis. Nat Rev Rheumatol (2025). https://doi.org/10.1038/s41584-025-01309-7

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