Advances in the pathophysiology, diagnosis and treatment of Takayasu arteritis
摘要
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.