Features of management of patients with pah associated with systemic connective tissue diseases in real clinical practice

The article deals with the problem of diagnosis of pulmonary arterial hypertension (PAH) in systemic connective tissue diseases. PAH is 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 features of the management of patients with PAH associated with systemic connective tissue diseases in real clinical practice are presented. A clinical case is presented. The importance of early diagnosis, careful differential diagnosis and its verification using invasive methods for assessing central hemodynamics is noted. Early initiation of the initial pathogenetic combination therapy based on a comprehensive assessment of the risk of mortality and disease progression can improve not only clinical symptoms, exercise tolerance, indicators of biochemical markers, but also slow down the progression of the disease, is the key to improving the prognosis in patients with PAH. Comprehensive management of patients by a team of cardiologist and rheumatologist allows improving the volume and quality of medical care, taking into account the individual characteristics of the patient, and applying a personalized approach to therapy.