Takayasu Arteritis

Abstract

Takayasu arteritis (TAK) remains an intractable disease with an unidentifiable cause involving inflammation of the aorta and its major branches, despite the many studies since the first case report by Dr. Mikito Takayasu in 1908. Although a rare disease, the estimated number of existing TAK patients is relatively large in Japan, a little over 6000. Female patients with early onset, the classic icon of TAK, still compose a major proportion; however, the proportions of male patients differ among countries. Even in current new Japanese patients, the male:female ratio has increased to 1 : 5. Presently, patients with elderly onset are also reported across the world. Patients in the initial stage of onset often complain of pains in various body regions and have systemic manifestations. Vascular lesions commonly involve branches of the aortic arch. Localized cervicobrachial involvements frequently occur in young female patients, whereas localized abdominal lesions tend to be observed in male patients. Current imaging techniques are useful for detecting signs of arterial inflammation prior to the development of a significant artery stenosis or dilatation. Nevertheless, surgery still has an important role for patients with critical vascular disorders. Surgical interventions, namely, endovascular treatment and open surgery, should ideally be avoided during the active phase of TAK owing to considerable risk of postinterventional failure. To achieve a favorable long-term outcome, an adequate anti-inflammatory treatment is essential. Optimal surgical management can improve life expectancy of patients. However, the high frequency of arterial restenosis after revascularization is still a significant problem in TAK. Restenosis occurs more frequently after endovascular treatment than after surgical bypass treatment generally. Based on these premises, and for assessing the immune status and the expected natural prognosis of the patient, the indication and strategy of vascular interventions should be carefully discussed by a multidisciplinary team.