Pulmonary arterial hypertension in systemic rheumatic diseases: patients management algorithms
Kanevskaya M.S.

The First Moscow State Medical University named after I.M. Sechenov

The article consideres the problem of diagnosis of pulmonary arterial hypertension in systemic connective tissue diseases. Pulmonary hypertension is a group of diseases and syndromes characterized by a progressive increase in pulmonary vascular resistance, which leads to the development of right ventricular heart failure and premature death of patients. The criteria of PH is the mean pulmonary artery pressure> 25 mm Hg at rest, detected by right heart catheterization. The definition and diagnostic criteria of pulmonary hypertension, data on the frequency of its detection in rheumatic diseases are presented. The need for early diagnosis, careful differential diagnosis and its verification using invasive methods for assessing central hemodynamics is indicated. Pulmonary arterial hypertension associated with systemic connective tissue diseases is a syndrome pathogenetically similar to idiopathic pulmonary arterial hypertension, but it evolves as a clinical manifestation of systemic connective tissue diseases, first of all, primarily systemic scleroderma. PAH-SCTD is included in group I according to the modern classification of pulmonary hypertension and according to the data of various registers is the first in frequency among all associated forms. The author presents a model for screening patients with systemic scleroderma and systemic connective tissue diseases, which simplifies the indications for the examination of patients. Modern approaches to drug therapy are described, as well as an algorithm for the appointment and conduct of PAH-specific therapy that improves the quality of life of patients and the survival rate for this very severe and prognostically unfavorable pathology.

Key words: pulmonary arterial hypertension, systemic connective tissue diseases, systemic scleroderma, PAH therapy, bosentan.
For citation: Kanevskaya M.S. Pulmonary arterial hypertension in systemic rheumatic diseases: patients management algorithms // RMJ. 2017. № 14. P.1026 –1032.