Lung function in idiopathic pulmonary fibrosis - extended analyses of the IFIGENIA trial

Ifigenia Study Grp, Juergen 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 LankhorstMarco Sardina, Gabrielle Boissard

Research output: Contribution to journalArticlepeer-review

Abstract / Description of output

Background: The 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.

Methods: We analysed effects of NAC on VC, DLco, a composite physiologic index (CPI), and mortality in the 155 study-patients.

Results: In 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.

Conclusion: This 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.

Original languageEnglish
Article number101
Pages (from-to)-
Number of pages9
JournalRespiratory research
Publication statusPublished - 27 Oct 2009


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