Behr, JürgenDemedts, MauritsBuhl, RolandCostabel, UlrichDekhuijzen, Richard P NJansen, Henk MMacNee, WilliamThomeer, MichielWallaert, BenoitLaurent, FrancoisNicholson, Andrew GVerbeken, Eric KVerschakelen, JohnyFlower, C D RPetruzzelli, StefanoDe Vuyst, Paulvan den Bosch, J M MRodriguez-Becerra, EulogioLankhorst, IdaSardina, MarcoBoissard, Gabrielle2014-06-262014-06-262009-10-27Behr 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; 10:1011465-9921http://hdl.handle.net/10668/1657Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't;Trial Registration: Registered at http://www.ClinicalTrials.gov; number NCT00639496.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.enAncianoAzatioprinaProgresión de la efermedadMétodo doble ciegoQuimioterapia combinadaEuropa (continente)Prueba de esfuerzoFemeninoHumanosPulmónFibrosis pulmonar IdiopáticaMediana edadPacientes desistentes del TratamientoPrednisonaFármacos del sistema respiratorioÍndice de severidad de la enfermedadResultado del tratamientoCapacidad vitalAcetilcisteínaMedical Subject Headings::Named Groups::Persons::Age Groups::Adult::AgedMedical Subject Headings::Chemicals and Drugs::Organic Chemicals::Sulfur Compounds::Thionucleosides::AzathioprineMedical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Disease Attributes::Disease ProgressionMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Research Design::Double-Blind MethodMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Therapeutics::Drug Therapy::Drug Therapy, CombinationMedical Subject Headings::Geographicals::Geographic Locations::EuropeMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Diagnostic Techniques, Cardiovascular::Heart Function Tests::Exercise TestMedical Subject Headings::Check Tags::FemaleMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::HumansMedical Subject Headings::Diseases::Respiratory Tract Diseases::Lung Diseases::Pulmonary Fibrosis::Idiopathic Pulmonary FibrosisMedical Subject Headings::Anatomy::Respiratory System::LungMedical Subject Headings::Named Groups::Persons::Age Groups::Adult::Middle AgedMedical Subject Headings::Named Groups::Persons::Patients::Patient DropoutsMedical Subject Headings::Chemicals and Drugs::Polycyclic Compounds::Steroids::Pregnanes::Pregnadienes::Pregnadienediols::PrednisoneMedical Subject Headings::Chemicals and Drugs::Chemical Actions and Uses::Pharmacologic Actions::Therapeutic Uses::Respiratory System AgentsMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Health Surveys::Health Status Indicators::Severity of Illness IndexMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Prognosis::Treatment OutcomeMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Diagnostic Techniques, Respiratory System::Respiratory Function Tests::Lung Volume Measurements::Total Lung Capacity::Vital CapacityMedical Subject Headings::Chemicals and Drugs::Organic Chemicals::Sulfur Compounds::Amino Acids, Sulfur::Cysteine::Acetylcysteineresearch article19860915open access10.1186/1465-9921-10-1011465-993XPMC2774307