Longterm treatment effects of pulmonary hypertantion (PH) in idiopathic pulmonary fibrosis (IPF) patients by endothelin receptor blockade (Bosentan)
M. Dimadi, F. P. Kokkinis, R. Trigidou, S. Kakouros, T. Raptakis, I. Tsagaridou, A. Vassilopoulos, D. Bouros (Athens, Lamia, Alexndroupoli, Greece)
Source: Annual Congress 2007 - Considerations in the management of idiopathic pulmonary fibrosis and other interstitial lung diseases
Disease area: Interstitial lung diseases, Pulmonary vascular diseases
Abstract Purpose: PH is a complication of IPF and contributes to the poor quality of life and the unfavorable survival. The role of Bosentan, a dual endothelin receptor antagonist, for the treatment in IPF-PH treatment has not been evaluated adequately. Methods: Prospective comparative study of 17 IPF patients with PH confirmed by ECHO (PASP >30 mmHg), NYHA/WHO classes II - IV, 6 minute walk distance test (6MWDT) and 12 months treatment. They classified into those treated with Bosentan (group A, n=7) and those without Bosentan (group B, n=10). They were checked every 3 months by ECHO, PFTs, ABGs and dyspnea stage. Results: The two groups did not differ at baseline in mean PASP (50.28 +/- 14.13 vs 44.4 +/- 12.86 p=0.4), mean % predicted PFTs and in NYHA/WHO class. A significant reduction of PASP (38.71 +/- 15.54, p=0,23 ) observed in group A at 3 months and at 6 months (34.75 +/-15.92) and remained stable up to 12 month. Dyspnoea stage and 6MWDT but not PFTs improved significantly during therapy. Survival in group A was 85.7% (1 patient died) vs 30% (7 patients died) in group B (HR=6.58, p=0.074). Advanced stage of dyspnoea inversely correlated to survival. Conclusions: Bosentan seems to be beneficial in patients with IPF-PH, but any impact of therapy needs further randomized evaluation in a large size of patients.
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M. Dimadi, F. P. Kokkinis, R. Trigidou, S. Kakouros, T. Raptakis, I. Tsagaridou, A. Vassilopoulos, D. Bouros (Athens, Lamia, Alexndroupoli, Greece). Longterm treatment effects of pulmonary hypertantion (PH) in idiopathic pulmonary fibrosis (IPF) patients by endothelin receptor blockade (Bosentan). Eur Respir J 2007; 30: Suppl. 51, 821
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