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Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases
  1. Christopher P Denton (c.denton{at}
  1. Royal Free Hospital, United Kingdom
    1. Janet E Pope (janet.pope{at}
    1. St. Joseph’s Healthcare, Canada
      1. Hans-Hartmut Peter (hans-hartmut.peter{at}
      1. Med. Universitätsklinik Freiburg, Germany
        1. Armando Gabrielli (a.gabrielli{at}
        1. Azienda Ospedaliera Umberto I, Italy
          1. Anco Boonstra (a.boonstra{at}
          1. Vrije Universteit Medisch Centrum, Netherlands
            1. Frank H. J. van den Hoogen (f.vandenhoogen{at}
            1. Universitair Medisch Centrum St. Radboud, Netherlands
              1. Gabriela Riemekasten (gabriela.riemekasten{at}
              1. Charité Universitätsmedizin, Germany
                1. Salvatore De Vita (salvatore.devita{at}
                1. Azienda Ospedaliero Universitaria, Italy
                  1. Adele Morganti (adele.morganti{at}
                  1. Actelion Pharmaceuticals Ltd., Switzerland
                    1. Ouali Berkani (ouali.berkani{at}
                    1. Actelion Pharmaceuticals Ltd., Switzerland
                      1. Loic Guillevin (loic.guillevin{at}
                      1. Hopital Cochin, France


                        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: Fifty-three patients with PAH related to connective tissue diseases (PAH-CTD) in WHO functional class III received bosentan 62.5 mg b.i.d. for 4 weeks and then 125 mg b.i.d. 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.

                        • Bosentan
                        • connective tissue diseases
                        • endothelin
                        • endothelin receptors
                        • pulmonary arterial hypertension

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