Long-Term Prospective Observation Study of Patients with Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases.

Aim    Analysis of survival and the impact of etiology, adverse prognosis factors, and therapy on the survival of patients with pulmonary arterial hypertension associated with immune-mediated inflammatory rheumatic diseases (PAH-IIRD).Material and methods    The study included 95 patients: 76 with systemic scleroderma (SSc), 9 with mixed connective tissue disease (MCTD), 8 with systemic lupus erythematosus (SLE), one with rheumatoid arthritis, and one with Sjogren's disease with diagnosed PAH. All patients were prescribed PAH-specific therapy and followed up for at least 5 years during this treatment. The endpoint of the study was all-cause death.Results    During the 5-year follow-up period, 37 patients with PAH-SSc and 4 with PAH-MCTD (43%) died. There were no fatal outcomes in PAH-SLE. One-, two-, three-, and five-year survival rates in the overall group of patients were 91%, 80%, 73%, and 57%, respectively. In patients with PAH-SSc, one-, two-, three-, and five-year survival rates were worse than in PAH-MCTD (88%, 76%, 68%, 51% and 100%, 89%, 89%, 56%, respectively). The factors associated with a fatal outcome included age, gender, functional class, 6-minute walk test distance, right atrial pressure, cardiac output, pulmonary vascular resistance, and biomarker (uric acid and N-terminal pro-brain natriuretic peptide) concentrations. The use of macitentan and/or riociguat, as monotherapy or in combination with another PAH-specific drug, significantly reduced the 5-year risk of fatal outcome (OR 0.38 [0.16; 0.89], p=0.027).Conclusion    The survival of patients with PAH-IIRD remains low. Further studies aimed at finding new pathogenetic targets are needed; the use of modern PAH-specific drugs (macitentan and/or riociguat) modifies the course of the disease, increasing the survival.