Long-term effects of bosentan in pulmonary arterial hypertension related to connective tissue diseases
Author Information
Author(s): 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
Primary Institution: Royal Free Hospital, London, UK
Hypothesis
This study investigated the long-term effects of bosentan in patients with pulmonary arterial hypertension related to connective tissue diseases.
Conclusion
Bosentan was associated with improvement or stability of clinical status in most patients, with a 92% survival estimate at 48 weeks.
Supporting Evidence
- 27% of patients improved in WHO class at week 48.
- 68% absence of clinical worsening at week 48.
- 92% survival rate at week 48.
Takeaway
The study looked at how a medicine called bosentan helps people with a serious lung problem related to their connective tissue diseases, and it found that most people did better or stayed the same after treatment.
Methodology
53 patients received bosentan for 48 weeks, with assessments of WHO class, quality of life, and survival.
Potential Biases
Potential for placebo effect influencing perceived improvements.
Limitations
The absence of a placebo group may limit the interpretation of the results.
Participant Demographics
{"mean_age":63,"gender_distribution":{"male":9,"female":44},"ethnicity":{"caucasian":51,"asian":2},"conditions":{"limited_systemic_sclerosis":29,"diffuse_systemic_sclerosis":13,"mixed_connective_tissue_disease":6,"systemic_lupus_erythematosus":5}}
Statistical Information
Confidence Interval
{"WHO_class_improvement":"95% CI 16–42%","clinical_worsening_absence":"95% CI 55–82%","survival":"95% CI 85–100%"}
Statistical Significance
p<0.05
Digital Object Identifier (DOI)
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