Beneficial effects of long-term treatment with bosentan on the development of pulmonary arterial hypertension in patients with systemic sclerosis

Objective To investigate the effects of long-term treatment with bosentan on pulmonary arterial hypertension (PAH) in patients with systemic sclerosis. Methods Patients with systemic sclerosis were followed between 2003 and 2014; those who developed digital ulcers were treated with standard regimens of bosentan. Patients were assessed at baseline and every 12months using transthoracic Doppler echocardiography, 6-min walking distance test, Borg dyspnoea index and monitoring of plasma levels of 76-amino-acid N-terminal probrain natriuretic peptide. Patients who developed PAH underwent right heart catheterization to confirm the diagnosis. Results Sixty-nine patients with systemic sclerosis were enrolled in the study. Of these, 25 developed digital ulcers and received treatment with bosentan; the remaining 44 comprised the control group. None of the patients treated with bosentan developed PAH during the follow-up period. Furthermore, in these patients the meanSD systolic pulmonary arterial pressure significantly decreased from 33.64 +/- 2.91mmHg at baseline to 26.20 +/- 1.78mmHg at the end of the follow-up period. In contrast, in the control group, seven patients developed PAH during the follow-up period, with the mean +/- SD systolic pulmonary arterial pressure significantly increasing from 33.57 +/- 2.75mmHg at baseline to 39.41 +/- 4.11mmHg at the end of the follow-up period. Conclusion Long-term treatment with bosentan reduces the risk of developing PAH in patients with systemic sclerosis.