Treatment Outcomes in Pulmonary Arterial Hypertension (PHA) Associated with Systemic Sclerosis (Sscl.) in Matched Groups Using Either Bosentan or Sildenafil Monotherapy

Abstract

Purpose The purpose of the study was to compare treatment outcomes in patients diagnosed with Pulmonary Arterial Hypertension (PAH) in association with Sscl. using Bosentan or Sildenafil monotherapy. Methods and Materials The Newcastle database was searched to identify all patients with (PAH) in association with Sscl. who had been treated at the Northern Pulmonary Vascular Service with either Bosentan or Sildenafil as monotherapy. The outcomes of two groups matched for age, haemodynamics, six minute walk (6MWD) and functional class at baseline who were treated with either Sildenafil or Bosentan monotherapy were compared at 3 and 6 months on therapy. Patients were selected who had no exercise limitation due to joint or muscle disease. Primary outcome measures were 6MWD and Camphor quality of life scores. Results A total of 25 patients were identified,12 treated with Sildenafil and 13 with Bosentan. In the Sildenafil group there was no significant differences in either Camphor or 6MWT at 0-3 or 0-6mths. Camphor scores, symptoms 0-3mths p= 0.33 0-6mths p=0.89, activites 0-3mths p-=0.94 0-6mths p=0.18, quality of life (QOL) 0-3mths p= 0.91, 0-6mths p=0.73. At baseline 6MWT mean (SD) 238(102) 3mths 252(126) 6mths 288(104) 0-3mths p=0.52, 0-6mths p=0.25. The Bosentan group showed a significant difference in 6MWT,baseline mean (SD) 233(126) 3mths 276(137) 6mths 264(104) 0-3mths p=0.01, 0-6mths p=0.02, however there were no significant differences in Camphor scores. Symptoms 0-3mths p=0.17, 0-6mths p=0.45, activities 0-3mths p=0.28, 0-6mths 0.08, QOL 0-3mths p=0.57, 0-6mths 0.34. Conclusions There was a significant improvement in 6MWD at 3 and 6 months seen in treatment with Bosentan but not Sildenafil. There were no improvements seen in Camphor scores in either group.