RT Journal Article T1 Lung function in idiopathic pulmonary fibrosis--extended analyses of the IFIGENIA trial. A1 Behr, Jürgen A1 Demedts, Maurits A1 Buhl, Roland A1 Costabel, Ulrich A1 Dekhuijzen, Richard P N A1 Jansen, Henk M A1 MacNee, William A1 Thomeer, Michiel A1 Wallaert, Benoit A1 Laurent, Francois A1 Nicholson, Andrew G A1 Verbeken, Eric K A1 Verschakelen, Johny A1 Flower, C D R A1 Petruzzelli, Stefano A1 De Vuyst, Paul A1 van den Bosch, J M M A1 Rodriguez-Becerra, Eulogio A1 Lankhorst, Ida A1 Sardina, Marco A1 Boissard, Gabrielle K1 Anciano K1 Azatioprina K1 Progresión de la efermedad K1 Método doble ciego K1 Quimioterapia combinada K1 Europa (continente) K1 Prueba de esfuerzo K1 Femenino K1 Humanos K1 Pulmón K1 Fibrosis pulmonar Idiopática K1 Mediana edad K1 Pacientes desistentes del Tratamiento K1 Prednisona K1 Fármacos del sistema respiratorio K1 Índice de severidad de la enfermedad K1 Resultado del tratamiento K1 Capacidad vital K1 Acetilcisteína AB BACKGROUNDThe randomized placebo-controlled IFIGENIA-trial demonstrated that therapy with high-dose N-acetylcysteine (NAC) given for one year, added to prednisone and azathioprine, significantly ameliorates (i.e. slows down) disease progression in terms of vital capacity (VC) (+9%) and diffusing capacity (DLco) (+24%) in idiopathic pulmonary fibrosis (IPF). To better understand the clinical implications of these findings we performed additional, explorative analyses of the IFGENIA data set.METHODSWe analysed effects of NAC on VC, DLco, a composite physiologic index (CPI), and mortality in the 155 study-patients.RESULTSIn trial completers the functional indices did not change significantly with NAC, whereas most indices deteriorated with placebo; in non-completers the majority of indices worsened but decline was generally less pronounced in most indices with NAC than with placebo. Most categorical analyses of VC, DLco and CPI also showed favourable changes with NAC. The effects of NAC on VC, DLco and CPI were significantly better if the baseline CPI was 50 points or lower.CONCLUSIONThis descriptive analysis confirms and extends the favourable effects of NAC on lung function in IPF and emphasizes the usefulness of VC, DLco, and the CPI for the evaluation of a therapeutic effect. Most importantly, less progressed disease as indicated by a CPI of 50 points or lower at baseline was more responsive to therapy in this study. PB BIOMED CENTRAL LTD SN 1465-9921 YR 2009 FD 2009-10-27 LK http://hdl.handle.net/10668/1657 UL http://hdl.handle.net/10668/1657 LA en NO Behr J, Demedts M, Buhl R, Costabel U, Dekhuijzen RP, Jansen HM, et al. Lung function in idiopathic pulmonary fibrosis--extended analyses of the IFIGENIA trial. Respir. Res. 2009; 10:101 NO Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't;Trial Registration: Registered at http://www.ClinicalTrials.gov; number NCT00639496. DS RISalud RD Apr 17, 2025