PULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC CONNECTIVE TISSUE DISEASES: THE CURRENT STATE OF THE PROBLEM

Pulmonary arterial hypertension (PAH) is a complex, multidisciplinary problem of modern medicine. The basis for the disease is a microcirculatory lesion in the vessels, which leads to a decrease in their blood flow, to increased pulmonary vascular resistance and, as an outcome, right ventricular failure, and death. PAH may also be associated with systemic connective tissue diseases (SCTDs), in particular with systemic sclerosis (SS), as one of the manifestations of the disease. As well as idiopathic pulmonary hypertension, PAH-SCTDs is an orphan disease: according to the data of the V.A. Nasonova Research Institute of Rheumatology, the incidence of PAH is 5.2% in SS, 0.3% in systemic lupus erythematosus, and 7.4% in mixed connective tissue disease. Low prevalence is responsible for late diagnosis, which is always associated with a poor therapy response and poor prognosis. The purpose of the lecture is to acquaint rheumatologists with the possibilities of diagnosis and treatment of this rare, but prognostic severe manifestation of SCTDs.