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Advances in the pathophysiology, diagnosis and treatment of Takayasu arteritis
Amr H Sawalha1, Durga P Misra2, Ruchika Goel3
1Departments of Pediatrics, Medicine, and Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. asawalha@pitt.edu.
Insights
Takayasu arteritis (TAK) is a rare large-vessel vasculitis affecting young women, targeting the aorta. Advances in imaging, biomarkers, and biologics are improving diagnosis and treatment for this complex inflammatory condition.
Area of Science:
- Cardiovascular Medicine
- Rheumatology
- Immunology
Background:
- Takayasu arteritis (TAK) is a rare, chronic, large-vessel vasculitis primarily affecting the aorta and its branches.
- Characterized by granulomatous inflammation, TAK predominantly impacts women aged 20-40, with significant geographic prevalence variations.
- Genetic susceptibility, particularly in immune-related genes, is implicated, but environmental factors in etiology remain unclear.
Purpose of the Study:
- To provide a comprehensive review of Takayasu arteritis for clinicians and researchers.
- To integrate recent advancements in understanding TAK pathogenesis, diagnosis, and management.
- To highlight evolving treatment strategies, including biologic agents for refractory cases.
Main Methods:
- Review of current literature on Takayasu arteritis.
- Focus on non-invasive imaging techniques for diagnosis and monitoring.
- Exploration of biomarker development for disease activity assessment.
Main Results:
- Non-invasive imaging is crucial for TAK diagnosis and monitoring.
- Biomarkers show potential for accurate disease activity assessment.
- Biologic agents offer new therapeutic avenues for refractory and relapsing TAK.
Conclusions:
- Understanding TAK pathogenesis is driving evolving management strategies.
- Integrated approaches using imaging, biomarkers, and novel therapies improve patient care.
- This review serves as a resource for advancing TAK knowledge and clinical practice.
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.
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