Principles of Diagnostics and Treatment of Patients with Takayasu Arteritis

Urgency of problem: Many types of vasculitis syndromes are rare and intractable diseases with unknown etiology and pathogenesis; these vasculitis are relatively prevalent and difficult to treat, among such diseases Takayasu Arteritis (TA). Takayasu Arteritis is a nonspecific large vessel vasculitis characterized by granulomatous inflammation of the vessel wall, predominantly with the damage of aorta, aortic arch and its major branches [Toyoshima S, et al. 2008; Keser G, et al. 2014]. In the initial stage of the disease, non-specific constitutional symptoms such as fever, malaise and weight loss may occur. Later, inflammation of the involved arteries progresses, resulting in segmental stenosis, occlusion, dilatation and/ or aneurysm. TA can show different patterns of arterial involvement, disease expression and prognosis in different regions of the world [Brunner J, et al. 2010]. So, there are no universal clinical recommendations and protocols of the TA managing. The purpose of our work was to evaluate results of medical and surgical treatment of TA in Uzbekistan (Asia) and offer algorithms of diagnostics, clinical management and treatment of TA. There were analyzed 210 patients with TA, young female patients were predominant (male/female ratio = 1:7.3; middle age = 32.1 ± 1.3 years); brachio-cephal arteries damage (100%). Combined lesion (aortic arch + thoracic or abdominal or renal arteries) was in 58% of patients. The coronary, renal arteries and pulmonary arteries also be involved in some patients. Many studies use a combination of clinical symptoms sometimes linked to changes in acute-phase reactants. We believe, that Duplex Scan (DS) is very important for detection of stenosis/occlusion of arteries and useful for evaluation of inflammation intensity in the complex with biochemical inflammatory markers such as C-reactive protein, leucocytes-count, RBS sedimentation velocity. To reach the main purpose of the medical treatment of TA- conversion of the acute phase of TA to morphological remission, glucocorticoid treatment is necessary. The typical initial dose is approximately 20 to 30 mg/day prednisolone (PSL), though the dose should be adjusted according to the age and constitution of the patient. Our investigation shows that in acute and subacute phase of TA disease prolonged using of glucocorticoid in “small” doses (10-30 mg/day) is less effective. We have received good results from pulse-therapy. Scheme of medical treatment is 1000 mg of cyclophosphamide and 1000 mg PSL intravenous at the first day, then, at the second and third days – 1000 mg