Pathophysiology, Diagnosis, and Management of Takayasu Arteritis: A Review of Current Advances

Abstract

Takayasu arteritis (TA) is a rare, chronic, inflammatory vasculitis that primarily affects large arteries, causing significant morbidity and mortality. This review provides an overview of the pathophysiology, diagnosis, and management of TA based on current advances in the field. TA is characterized by autoimmune-mediated inflammation, vascular remodeling, and endothelial dysfunction. The disease progresses through three stages (active, chronic, and healing phase) each presenting distinct clinical features. Diagnosis of TA can be challenging due to non-specific clinical manifestations and the lack of specific diagnostic tests. Various imaging modalities, such as angiography, ultrasound, and Doppler techniques, play a crucial role in the diagnosis of TA by visualizing arterial involvement and assessing disease extent. Management of TA involves a multidisciplinary approach, with disease-modifying anti-rheumatic drugs (DMARDs) as the cornerstone of medical therapy. Synthetic and biologic DMARDs are used to induce remission, control inflammation, and prevent complications. Non-pharmacologic interventions, such as resistance exercises and curcumin supplementation, show potential benefits. Invasive interventions, including endovascular therapy and open surgery, are used for managing vascular lesions. However, challenges remain in disease understanding and management, including the heterogeneity of disease presentation and the lack of standardized treatment guidelines. The future of TA management lies in precision medicine, utilizing biomarkers and molecular profiling to personalize treatment approaches and improve patient outcomes. Further research is needed to unravel the underlying mechanisms of TA and develop targeted therapies.

Keywords: pathogenesis; review; rheumatology; takayasu arteritis; vascular.

Figure 1. Active phase of TA in descending thoracic aorta.

The images show (a) granulomatous inflammation at the medio-adventitial junction of aorta destroying the structure of an intercostal artery, (b) chronic phase of TA in descending thoracic aorta showing media (M) at the outer third with clusters of mononuclear cells. Note marked fibrosis of the adventitia (A) (hematoxylin and eosin, ×250). (c) The cells are lymphocytes, and few plasma cells and histiocytes (hematoxylin and eosin, ×250). TA: Takayasu arteritis The images are used with permission from Vaideeswar and Deshpande (2013) [5].

Figure 2. Healed phase of TA in descending thoracic aorta.

The images show (a) loose fibrocellular thickening of the intima (I), (b) the junctional area between intima (I) and media (M) showing the destruction of the elastic lamellae/smooth muscle with replacement fibrosis, (c) similar features are also seen toward the outer third of the media (M) and adventitia (A), (d) fibrotic adventitia (A) showing endarteritis obliterans of a branch (elastic van Gieson, ×250). TA: Takayasu arteritis The images are used with permission from Vaideeswar and Deshpande (2013) [5].