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 12 months 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 mean ± SD systolic pulmonary arterial pressure significantly decreased from 33.64 ± 2.91 mmHg at baseline to 26.20 ± 1.78 mmHg 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 ± 2.75 mmHg at baseline to 39.41 ± 4.11 mmHg at the end of the follow-up period.


CONCLUSIONS
Long-term treatment with bosentan reduces the risk of developing PAH in patients with systemic sclerosis.
Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Genoa, Italy Giuseppe.Murdaca@unige.it.
Giuseppe
G
Murdaca
Department of Health Sciences, Biostatistics Unit, University of Genoa, Genoa, Italy.
Francesca
F
Lantieri
Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Genoa, Italy.
Francesco
F
Puppo
Department of Internal Medicine, Division of Cardiology, University of Genoa, Genoa, Italy.
Gian Paolo
GP
Bezante
Department of Internal Medicine, Division of Cardiology, University of Genoa, Genoa, Italy.
Manrico
M
Balbi
Bosentan; endothelial dysfunction; pulmonary arterial hypertension; systemic sclerosis
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