• Jürgen Behr, Maurits Demedts, Roland Buhl, Ulrich Costabel, Richard P N Dekhuijzen, Henk M Jansen, William MacNee, Michiel Thomeer, Benoit Wallaert, Francois Laurent, Andrew G Nicholson, Eric K Verbeken, Johny Verschakelen, C D R Flower, Stefano Petruzzelli, Paul De Vuyst, J M M van den Bosch, Eulogio Rodriguez-Becerra, Ida Lankhorst, Marco Sardina, Gabrielle Boissard, and IFIGENIA study group.
• Medizinische Klinik I, Klinikum Grosshadern der Ludwig Maximilians-Universität, München, Germany. juergen.behr@med.uni-muenchen.de
• Resp Res. 2009 Jan 1;10:101.

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.

36 keywords...

hide…