The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology
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Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option. This study aimed to assess the impact of sequential combination therapy on both short-term responses and long-term outcomes in a real-world setting. Patients with idiopathic/heritable pulmonary arterial hypertension, or pulmonary arterial hypertension associated with congenital heart disease or connective tissue disease and who were not meeting treatment goals on either first-line bosentan or sildenafil monotherapy, were given additional sildenafil or bosentan and assessed after 3-4 months. ⋯ The 1-, 3- and 5-year overall survival estimates were 91%, 69% and 59%, respectively. Patients with pulmonary arterial hypertension associated with connective tissue disease had significantly poorer survival rates compared to other aetiologies (p<0.003). The favourable short-term haemodynamic results and good survival rates, observed in patients receiving both bosentan and sildenafil, supports the use of sequential combination therapy in patients failing on monotherapy in a real-world setting.
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Editorial Comment
Telomeres revisited: RTEL1 variants in pulmonary fibrosis.
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Editorial Historical Article
A tale of two global emergencies: tuberculosis control efforts can learn from the Ebola outbreak.