Pulmonary arterial hypertension associated with systemic sclerosis: Results of a single-center long-term follow-up in 2009–2024

The aim of this study was to investigate the survival and the impact of various factors on it in patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (PAH-SSc). Methods . We analyzed the data of 76 patients diagnosed with PAH-SSc who received PAH-specific therapy and were followed-up for at least 5 years. A group of “historical control” consisted of 20 patients who did not receive PAH-specific treatment. The primary endpoint of the study was death from any cases. Results . The use of PAH-specific therapy significantly reduces the 5-year risk of death in patients with PAH-SSc by 67%, compared to the “historical control” group. At the present time, 1-, 2-, 3-, and 5-year survival rates for patients with PAH-SSc are 88%, 76%, 68%, and 51%, respectively. Factors associated with mortality include age, functional class, 6-minute walking distance, right atrial pressure, cardiac output, pulmonary vascular resistance, and elevated biomarkers. The use of macitentan and/or riociguat as monotherapy or in combination with other PAH-specific medications significantly reduced the risk of death after 5 years (hazard ratio – 0.44 [0.19; 1.07]; p =0.07). However, immunosuppressive therapy did not improve survival. Conclusion . The survival rate for patients with PAH-SSc remains low. Further research is needed to identify new treatments targets. The use of modern PAH-specific drugs, such as macitentan and riociguat, can modify the course of disease and improve survival.