PAH-specific therapy reduced 5-year mortality risk by 67% versus historical controls, and macitentan or riociguat use trended toward reduced death risk (HR 0.44; 95% CI 0.19-1.07; p=0.07).
Cohort (n=96)
No
Does PAH-specific therapy improve survival in patients with pulmonary arterial hypertension associated with systemic sclerosis?
PAH-specific therapy, including modern drugs like macitentan and riociguat, improves long-term survival in patients with systemic sclerosis-associated pulmonary arterial hypertension compared to historical controls.
Effect estimate: HR 0.44 (95% CI 0.19-1.07)
p-value: p=0.07
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.
Волков et al. (Wed,) conducted a cohort in Pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc) (n=96). PAH-specific therapy (including macitentan and/or riociguat) vs. Historical control (no PAH-specific treatment) was evaluated on Death from any cases (HR 0.44, 95% CI 0.19-1.07, p=0.07). PAH-specific therapy reduced 5-year mortality risk by 67% versus historical controls, and macitentan or riociguat use trended toward reduced death risk (HR 0.44; 95% CI 0.19-1.07; p=0.07).