RT Journal Article
SR Electronic
T1 Effect of bosentan on MMP-7 levels as add-on therapy in idiopathic pulmonary fibrosis. A prospective study
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p663
VO 38
IS Suppl 55
A1 Katerina Samara
A1 Evi Vassalou
A1 Ioannis Giannarakis
A1 Ismini Lasithiotaki
A1 Nikolaos Tzanakis
A1 Nikolaos Siafakas
A1 Katerina Antoniou
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p663.abstract
AB Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal lung disease lacking effective treatment. Endothelin receptor antagonists were shown to have possible beneficial effects in early stages of IPF. Recent evidence suggests that increased MMP7 levels may be indicative of disease progression.Objectives: To determine the effects of bosentan on MMP-7 serum levels in patients with IPF.Methods: We prospectively studied eleven IPF patients, nine males and two females, aged 68.9±8.8 years. Five were current or ex-smokers (43±23.5 pack/years), the remaining non-smokers. All patients received oral bosentan 62.5 mg twice daily for 4 weeks, increased to 125 mg twice daily thereafter, for 6 months or longer, as add-on therapy to 10mgr prednisolone, acetylcysteine (600 mg tid) and Azathioprine (150 mg/day). Serum MMP-7 levels were measured using commercial ELISA kits. All the patients had routine laboratories, PFTs and radiological (HRCT) tests, 6-minute walking distance (6MWD) and alveolar-arterial gradient of oxygen tension (PA-aO2) measured at baseline, three and six months of treatment.Results: Serum MMP-7 levels did not show any significant change from baseline (6.4±3.22 pg/ml) to three (7.39±4.77 pg/ml) and 6 months (7.61±4.76 pg/ml). There were no significant alterations in FVC, DLCO, 6MWD and PA-aO2, pre- and post-treatment. No cases of clinically significant infection, leucopenia, elevated liver enzymes or other unexpected adverse events were reported.Conclusions: The above data suggest that bosentan as an add-on agent to standard therapy is a safe therapeutic modality which results in disease stabilization as assessed by serum MMP-7 and functional status.