Takayasu arteritis (TA) is a systemic vasculitis with predominatly lesions of aorta and its large branches. In some cases pulmonary arteries (PA) are involved in the pathological inflammatory process and lead to the formation of pulmonary hypertension and significantly worse the prognosis. Timely development of lesion of PA, appointment of adequate therapy and surgical treatment can prevent irreversible damage of blood vessels and improve the prognosis. Perioperative administration of interleukin-6 inhibitor inhibitor (tocilizumab) in at patients with indications for vascular surgery, including angioplasty PA, should be considered as a promising approach to control the inflammatory activity of TA, reduce the dose of glucocorticoids and the risk of postoperative complications. We present the clinical experience of significant improvement in the patients condition was achieved by using two-stage balloon angioplasty on the background of control of the disease activity with interleukin-6 tocilizumab intravenously and specific therapy with riociguat and iloprost.