Abstract
Idiopathic Pulmonary Fibrosis (IPF) is a progressive and fatal disease for which there are no currently effective therapies to improve patient outcomes and slow the progression of the disease. Recent clinical trials provided encouraging data on the use of pirfenidone in patients with mild -to-moderate IPF.
Fourteen 14 patients (mean age etc..) with moderate IPF were randomly treated with Pirfenidone + Acetylcysteine (group 1; 7 patients , 4male , mean age 63.3 ± 13 , 2 former-smokers / 5 non-smokers; functional data: FVC 2.14 L ( 59.75 % th ) , DLCO 43% , 6MWT mt 474 ± 115) or with Acetlcysteine alone (group 2; 7 patients, 6 male, mean age 66.1 ± 11 , 3 former-smokers / 4 non-smokers; FVC 1.99 L ( 64.7% th ) , 46% DLCO , 6MWT paths 482 mt ± 138). The patients were followed as outpatients for one year.
Results: After 12 months, respiratory functional data documented reduced FVC (-5 and -3 relative percent respectively in group 1 and 2 , chi2 -test p = 0.8), DLCO (-8 and -7 relative percent difference respectively in group 1 and 2, p = 0.9 chi2 -test ) as well as reduced distance walked in meters. (- 45 and -50 m paths respectively in group 1 and 2, chi2 -test p = 0.9 ).
Conclusions: Combined therapy with pirfenidone and acetylcysteine in patients with moderate IPF is well tolerated but did not have significant effects on functional outcomes compared with acetylcysteine treatment in monotherapy.
- © 2014 ERS
