Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases

被引：106

作者：

Denton, C. P. ^{[1
]}

Pope, J. E. ^{[2
]}

Peter, H-H ^{[3
]}

Gabrielli, A. ^{[4
]}

Boonstra, A. ^{[5
]}

van den Hoogen, F. H. J. ^{[6
]}

Riemekasten, G. ^{[7
]}

De Vita, S. ^{[8
]}

Morganti, A. ^{[9
]}

Doelberg, M. ^{[9
]}

Berkani, O. ^{[9
]}

Guillevin, L. ^{[10
]}

机构：

[1] Royal Free Hosp, Ctr Rheumatol, Rheumatol Unit, London NW3 2QG, England

[2] St Josephs Healthcare, London, ON, Canada

[3] Med Univ Klin Freiburg, Freiburg, Germany

[4] Azienda Osped Umberto 1, Ancona, Italy

[5] Vrije Univ Med Ctr, Amsterdam, Netherlands

[6] Radboud Univ Nijmegen Med Ctr, Nijmegen, Netherlands

[7] Charite, D-13353 Berlin, Germany

[8] Azienda Osped Univ, Rheumatol Clin, DPMSC, Udine, Italy

[9] Actel Pharmaceut Ltd, Allschwil, Switzerland

[10] Hop Cochin, F-75674 Paris, France

来源：

ANNALS OF THE RHEUMATIC DISEASES | 2008年 / 67卷 / 09期

关键词：

D O I：

10.1136/ard.2007.079921

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：1222 / 1228

页数：7

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