Sergio Harari1, Antonella Caminati1, Marco Confalonieri2, Venerino Poletti3,4, Carlo Vancheri5, Alberto Pesci6, Paola Rogliani7, Fabrizio Luppi8, Carlo Agostini9, Paola Rottoli10, Alessandro Sanduzzi Zamparelli11, Alfredo Sebastiani12, Rossana Della Porta2, Francesco Salton2, Barbara Messore13, Sara Tomassetti3, Roberta Rosso5, Alice Biffi6, Ermanno Puxeddu7, Stefania Cerri8, Francesco Cinetto9, Rosa Metella Refini10, Marialuisa Bocchino11, Loreta Di Michele12, Claudia Specchia14,15, Carlo Albera13. 1. U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare. Ospedale San Giuseppe - MultiMedica, IRCCS, Milano, Italy. 2. Department of Pulmonology, University Hospital of Cattinara, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy. 3. U.O. di Pneumologia Dipartimento dell'Apparato Respiratorio e del Torace, Ospedale G.P. Morgagni -L. Pierantoni, Forlì, Italy. 4. Department of Respiratory Diseases & Allergy, Aarhus University Hospital (DK), Aarthus, Denmark. 5. Regional Referral Centre for Rare Lung Disease, University of Catania, A.O.U. Policlinico-Vittorio Emanuele, Catania, Italy. 6. Respiratory Unit, Department of Health Science, University of Milano Bicocca, AO San Gerardo, Monza, Italy. 7. Respiratory Unit Policlinico Tor Vergata, Department of "Systems Medicine, University of Rome "Tor Vergata", Roma, Italy. 8. Center for Rare Lung Diseases, University Hospital Policlinico di Modena, Modena, Italy. 9. Department of Medicine - DIMED, University of Padova, Padova, Italy. 10. Respiratory Diseases and Lung Transplant Unit, Department of Internal and Specialist Medicine, AOUS, Siena, Italy. 11. UOC II Pneumotisiologia, Scuola di specializzazione in malattie respiratorie Università degli Studi di Napoli Federico II A.O.R.N. Monaldi-Cotugno-CTO Piazzale Ettore Ruggieri, Napoli, Italy. 12. UOS Interstiziopatie Polmonari Az Osp. S. Camillo-Forlanini, Roma, Italy. 13. Department of Clinical and Biological Sciences, Interstitial and Rare Lung Disease Unit AOU San Luigi Gonzaga, Orbassano, University of Turin, Turin, Italy. 14. Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy. 15. IRCCS MultiMedica, Milano, Italy.
Abstract
INTRODUCTION: Gender, age, physiology (GAP) system have proven to be an easy tool for predicting disease stages and survival in idiopathic pulmonary fibrosis (IPF) patients. OBJECTIVE: To validate mortality risk as determined by the GAP system in a real-life multicentre IPF population treated with pirfenidone. METHODS: The study included patients who received pirfenidone for at least 6 months. The GAP calculator and the GAP index were determined. The primary outcome was all-cause mortality. The prognostic accuracy of the GAP system was evaluated with respect to calibration and discrimination. RESULTS AND CONCLUSION: Sixty-eight IPF patients were enrolled in the study. The median follow-up was 2.4 years (range 0.1-7.4 years). A total of 22 deaths as first event (32%) and of 10 lung transplantation (15%) were recorded. The cumulative incidence of mortality at 1, 2 and 3 years was 10.4%, 22.4% and 38.4%, respectively. The differences between the predicted and observed mortality were not significant for the GAP index while the observed mortality become comparable to that predicted by the GAP calculator only in the third year of follow-up. The C-index for the GAP index was 0.74 (95% CI 0.57-0.93) while the C-statistic value for the GAP calculator was 0.77 (95% CI 0.59-0.95).
INTRODUCTION: Gender, age, physiology (GAP) system have proven to be an easy tool for predicting disease stages and survival in idiopathic pulmonary fibrosis (IPF) patients. OBJECTIVE: To validate mortality risk as determined by the GAP system in a real-life multicentre IPF population treated with pirfenidone. METHODS: The study included patients who received pirfenidone for at least 6 months. The GAP calculator and the GAP index were determined. The primary outcome was all-cause mortality. The prognostic accuracy of the GAP system was evaluated with respect to calibration and discrimination. RESULTS AND CONCLUSION: Sixty-eight IPF patients were enrolled in the study. The median follow-up was 2.4 years (range 0.1-7.4 years). A total of 22 deaths as first event (32%) and of 10 lung transplantation (15%) were recorded. The cumulative incidence of mortality at 1, 2 and 3 years was 10.4%, 22.4% and 38.4%, respectively. The differences between the predicted and observed mortality were not significant for the GAP index while the observed mortality become comparable to that predicted by the GAP calculator only in the third year of follow-up. The C-index for the GAP index was 0.74 (95% CI 0.57-0.93) while the C-statistic value for the GAP calculator was 0.77 (95% CI 0.59-0.95).