PT  - JOURNAL ARTICLE
AU  - C P Denton
AU  - J E Pope
AU  - H-H Peter
AU  - A Gabrielli
AU  - A Boonstra
AU  - F H J van den Hoogen
AU  - G Riemekasten
AU  - S De Vita
AU  - A Morganti
AU  - M Dölberg
AU  - O Berkani
AU  - L Guillevin
AU  - (on behalf of the TRacleer Use in PAH associated with Scleroderma and Connective Tissue Diseases (TRUST) Investigators)
TI  - Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases
AID  - 10.1136/ard.2007.079921
DP  - 2008 Sep 01
TA  - Annals of the Rheumatic Diseases
PG  - 1222--1228
VI  - 67
IP  - 9
4099  - http://ard.bmj.com/content/67/9/1222.short
4100  - http://ard.bmj.com/content/67/9/1222.full
SO  - Ann Rheum Dis2008 Sep 01; 67
AB  - Objectives: This study investigated the long-term effects of bosentan, an oral endothelin ETA/ETB 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.