Session Speaker

Efficacy of Bosentan in the Treatment of Advanced Pulmonary Fibrosis
J. Morera and J. Roldan
Spain


Introduction: Idiopathic Pulmonary Fibrosis (IPF) is an important form of progressive lung fibrosis that results in respiratory failure and death in the majority of patients within five years of diagnosis.

The association between Pulmonary Hypertension (PH) and IPF is common and is associated with poor prognosis. So far there is no treatment that has been proven effective. Bosentan is a dual and selective endothelin receptor antagonist, shown to have anti-proliferative, vasodilators, anti-inflammatory and anti-fibrotic effects. The objective was to evaluate whether treatment with Bosentan offers clinical benefit in patients with IPF and PH-related.

Methods: Prospective observational study in a cohort of 10 IPF patients with associated PH, treated with Bosentan in all cases were assessed the following variables at the beginning and after 1 year of follow-up. Evolution of walking test 6 minutes (WT6´) in meters, initial Dyspnea (VASI) and final test (VASF) with the WT6´ according to visual analog scale from 0 to 10, class functional NYHA, pulmonary arterial pressure (PAP) in mm Hg measured by Echocardiogram, St. George Respiratory Questionnaire quality of life (SGRQ), from 0 to 100 points, in which high scores indicate a lower quality of life. Follow-up that included monthly visits was performed with blood analysis of hepatic function.

Results: Diagnose of IPF was made it by chest CT scan, and in all the cases honeycomb type affectation was upper that 50 %. The mean age was 66, 6±12, 66 years, 60 % were men. One of them could not perform the WT6´ for inability to walk; two patients were exitus during the follow-up. After treatment with Bosentan, statistically significant improvement was appreciated in the distance traveled at the WT6´ (median value at entry 300 meters and 380 meters at the end of the one year-follow up (Wilcoxon test; p = 0,028). The median PAP value also improved (from 59 to 39mmHg) (Wilcoxon test, p =0,010) and in the NYHA functional classification 3 patients on class IV improved to class III, a patient improved from class IV to class II and 3 patients did not change (marginal; homogeneity test p=0,034).

A trend is appreciated for the SGRQ, VASI and VASF parameters toward improvement in all of them, without reaching statistical significance. No adverse effects were associated with Bosentan.

Conclusions: In this series, Bosentan therapy has been associated with a clinical improvement in functional capability of patients with advanced IPF and PH associated. WE believe that although future clinical trials are necessary,the use of inhibitors ET1 should be considered in the treatment of these patients.