Takayasu Arteritis

Takayasu arteritis (TKA) is a primary systemic large-vessel vasculitis involving the aorta and its major branches, usually affecting young females. Vascular lesions are typically stenotic or occlusive, and relapses tend to be frequent. Clinical presentation is often heterogeneous with constitutional and musculoskeletal symptoms, and ischemic manifestations derived from the involvement of arteries of extremities and other territories. Aortic arch aneurysm rupture and congestive cardiac failure due to aortic insufficiency are the two main causes of death. Uncontrolled or unrecognized hypertension is associated with poor long-term cardiovascular prognosis. Erythrocyte sedimentation (ESR) levels can be misleading in TKA because of the lack of correlation with the progression of vascular stenoses. Among vascular imaging techniques in TKA, magnetic resonance imaging (MRI) and MR-angiography (MRA) study is considered the most useful test for initial diagnosis and routine sequential follow-up. Disease activity is assessed by the combination of clinical manifestations, elevated ESR, and changes in vascular imaging techniques. The treatment of choice is based on glucocorticoids and additional immunosuppressive agents, preferably methotrexate. However, in patients with TKA refractory to these first-line agents, tocilizumab and anti-TNF agents have been demonstrated to be good therapeutic options. Revascularization procedures should be performed when disease is quiescent (if possible). Among them, bypass with autologous vessel grafts has shown better long-term patency results than angioplasty and stent placement. The 1994 topographic classification of vascular lesions at angiography, the 2010 European League Against Rheumatism (EULAR)/Paediatric Rheumatology European Society (PRES)/Paediatric Rheumatology International Trials Organisation (PRINTO), and the latest 2022 American College of Rheumatology (ACR)/EULAR classification criteria, are all supportive in assessing diagnosis and disease extent.