@article {Joglekar61, author = {Amit Joglekar and Fausan S Tsai and Deborah A McCloskey and Julianne E Wilson and James R Seibold and David J Riley}, title = {Bosentan in pulmonary arterial hypertension secondary to scleroderma.}, volume = {33}, number = {1}, pages = {61--68}, year = {2006}, publisher = {The Journal of Rheumatology}, abstract = {OBJECTIVE:. To assess the efficacy and tolerability of bosentan in pulmonary arterial hypertension secondary to systemic sclerosis (SSc-PAH) including patients with restrictive lung disease. METHODS: We retrospectively reviewed 23 SSc-PAH patients with PAH at baseline [PA systolic pressure (PASP) \>or= 45 mm Hg by echocardiogram or mean PA pressure \> 25 mm Hg at rest by cardiac catheterization], World Health Organization (WHO) functional classes II-IV, and with data available for 18 months. Bosentan dose was 62.5 mg twice daily for 1 month then 125 mg twice daily. Outcomes were WHO functional class, PASP, and pulmonary function tests (PFT) at 3-month intervals for 18 months. RESULTS: WHO class at baseline 3.1 +/- 0.1 (mean +/- SE); 3 months, 2.5 +/- 0.2*; 6 months, 2.4 +/- 0.2*; 9 months, 2.5 +/- 0.2* (*p \< 0.02 vs baseline, n = 21 to 23), indicating clinical improvement at 9 months. After 9 months, results were not significant versus baseline. Reduction in WHO class by at least one rank was 57\% at 3 months; none worsened. After 9 months, WHO class tended to worsen compared to baseline. Baseline PASP was 54 +/- 2 mm Hg (n = 23) and did not change significantly with therapy. Restriction (total lung capacity 76\% +/- 4\% of predicted) and reduced diffusing capacity (39\% +/- 3\% of predicted) were unchanged during therapy. Abnormal transaminases in 2 patients (9\%) necessitated discontinuing drug in both. CONCLUSION: Bosentan is clinically beneficial in patients with SSc-PAH including patients with restrictive lung disease, but pulmonary hemodynamics and PFT results remained stable during treatment.}, issn = {0315-162X}, URL = {https://www.jrheum.org/content/33/1/61}, eprint = {https://www.jrheum.org/content/33/1/61.full.pdf}, journal = {The Journal of Rheumatology} }