Abstract
Takayasu arteritis is a chronic, progressive, idiopathic, large-vessel vasculitis that affects the aorta, its main branches and the pulmonary arteries. It typically occurs in young Asian women but can be found in any ethnic group and in men. The disease is named after Mikito Takayasu, a Japanese ophthalmologist, who first described the arteriovenous anomalies in the retina of a patient with the disease in 1908. The etiopathogenesis is not known, but studies are being conducted regarding the immunological, infection and genetic aspects of the disease. Early during the course of the disease, inflammation of the involved arteries progresses, resulting in segmental stenosis, occlusion, dilatation and/or aneurysm. The clinical presentation of Takayasu arteritis varies depending on the blood vessels involved. Early symptoms are nonspecific, making the diagnosis difficult. Subsequently, arterial occlusions occur, producing more specific ischemic symptoms. Paucity of specific symptoms and laboratory biomarkers, as well as difficulties in assessing the disease activity and progression, make it often unrecognized at onset, and its activity is frequently underestimated. The diagnosis is usually confirmed by a combination of clinical manifestations, laboratory markers, diagnostic criteria and imaging methods. The purpose of this review is to address the current knowledge on pathogenesis, investigations, classification and management, and to emphasize the need for timely diagnosis, effective therapeutic intervention, and close monitoring of this disease.