Early therapy with bosentan may reverse pulmonary vasculopathy in scleroderma patients

Aims: Out-of-proportion pulmonary arterial pressure (PAP) increase during exercise may represent an intermediate stage between normal pulmonary hemodynamics and manifest PAH and might precede PAH in systemic sclerosis (SSc). We hypothesised that bosentan treatment might reverse a trend towards progressive pulmonary vasculopathy in such patients. Patients and methods: SSc patients with a resting PAP30mmHg during exercise underwent 3 right heart catheterisations (RHC) with hemodynamic measurements at rest, 50 W, and maximal exercise. Between the first and second RHC there was a 12 month control period. Then bosent…