Abstract
OBJECTIVE: To evaluate the clinical effects of adding inhaled iloprost to sildenafil for the treatment of patients with connective tissue disease (CTD)-associated PAH.
MATERIAL AND METHODS: Consecutive patients with connective tissue disease-associated PAH who received sildenafil for at least three months were enrolled and started on iloprost inhalation. At the end of six months, the patients were reassessed for changes in their New York Heart Association (NYHA) functional class, sixminute walk distance (6MWD), plasma brain natriuretic peptide (BNP) level, and their quality of life as assessed using the short form 36 (SF-36) health survey questionnaire.
RESULTS: The study included 27 patients with a mean age of 46.3 +/- 14.5 years. Scleroderma was most common primary diagnosis in 37% of cases. In the 23 patients who completed the study, NYHA class improved from 2.9 +/- 0.5 to 2.1 +/- 0.5 (p=0.027). Their 6MWD increased by 31% (262.1 +/- 126.2 m vs. 342.9 +/- 110.6 m; p<0.001). Post-exercise oxygen saturation increased from 85.9 +/- 9.1% to 90.4 +/- 5.9% (p<0.0001). The SF-36 score for general health and role limitation due to physical problems improved significantly. No difference in BNP was noted.
CONCLUSION: The addition of iloprost to sildenafil for treatment of connective tissue disease-associated PAH resulted in significant improvement in NHYA functional class, 6MWD and quality of life.