| Abstract | OBJECTIVES: This study investigated the long-term effects of bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, in patients with pulmonary arterial hypertension (PAH) exclusively related to connective tissue diseases (CTD). METHODS: A total of 53 patients with PAH related to connective tissue diseases (PAH-CTD) in World Health Organization (WHO) functional class III received bosentan 62.5 mg twice a day for 4 weeks and then 125 mg twice a day for 44 weeks in this open non-comparative study. Assessments at weeks 16 and 48 included WHO class, clinical worsening, quality of life (Short-Form Health Survey (SF-36) and health assessment questionnaire (HAQ) modified for scleroderma), and survival (week 48 only). Safety and tolerability were monitored throughout the study. RESULTS: At week 48, WHO class improved in 27% of patients (95% CI 16-42%) and worsened in 16% (95% CI 7-29%). Kaplan-Meier estimates were 68% (95% CI 55-82%) for absence of clinical worsening and 92% (95% CI 85-100%) for survival. Overall changes in quality of life were minimal. There were no unexpected side effects observed during the study. CONCLUSIONS: In most patients, bosentan was associated with improvement or stability of clinical status. The 92% estimate for survival at 48 weeks is a significant achievement in this patient population. |
| Authors | C P Denton, J E Pope, H-H Peter, A Gabrielli, A Boonstra, F H J van den Hoogen, G Riemekasten, S De Vita, A Morganti, M Dölberg, O Berkani, L Guillevin, TRacleer Use in PAH associated with Scleroderma and Connective Tissue Diseases (TRUST) Investigators
(Affiliation: Center for Rheumatology, Royal Free Hospital, Pond Street, Rheumatology Unit, Lower Ground Floor, London NW3 2QG, UK. c.denton at medsch.ucl.ac.uk)
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| Journal | Annals of the rheumatic diseases
(Ann Rheum Dis)
Vol. 67
Issue 9
Pg. 1222-8
(Sep 2008)
ISSN: 1468-2060 England |
| PMID | 18055477
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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