Pulmonary Hypertension Associated with Systemic Sclerosis (Review)

Pulmonary hypertension (PH) is one of the leading causes of morbidity and mortality in systemic scleroderma (SSc). Objective — to analyse the current literature data on the epidemiology, mechanisms of development, risk, clinical manifestations, diagnosis, treatment and prognosis of patients with SSc with PH of different groups. Materials and methods. The literature analysis was conducted over the past 15 years (from 2010 to 2025) in the search databases Scopus, MEDLINE, PubMed. For the final analysis, 71 articles published in English were selected. Results and discussion. PH is diagnosed in 7—31 % of patients with SSc. Different groups of PH can develop in SSc: pulmonary arterial hypertension (PAH) associated with сonnective tissue diseases, PAH with features of venous/capillary involvement, PH associated with left heart disease, PH associated with lung disease and chronic thromboembolic PH. PAH develops in 12.9—19.7 % of patients and, despite targeted therapy, remains the leading cause of mortality in patients with SSc. The diagnosis of PH can be difficult due to the heterogeneity of clinical manifestations of SSc and the nonspecific clinical picture of PH in the early stages, which leads to late diagnosis. For the purpose of timely diagnosis, screening algorithms for early stage PAH have been developed, in particular, the DETECT and ASIG algorithms, to select patients for right heart catheterisation. Conclusions. PH is a severe manifestation/complication of SSc, which is associated with a poor prog­nosis and deterioration in the quality of life of patients. PH in SSc is a heterogeneous condition that includes PH of different groups. Early diagnosis and treatment of PH improves the prognosis of these patients. Screening for PAH is recommended for patients with SSc, as its early detection will lead to a reduction in haemodynamic disorders and improved survival.