1. The prognostic role of Gender-Age-Physiology system in idiopathic pulmonary fibrosis patients treated with pirfenidone
- Author
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Roberta Rosso, Fabrizio Luppi, Stefania Cerri, Carlo Albera, Ermanno Puxeddu, Alessandro Sanduzzi Zamparelli, Sergio Harari, Claudia Specchia, Alberto Pesci, Paola Rottoli, Marialuisa Bocchino, Venerino Poletti, Barbara Messore, Rosa Metella Refini, Carlo Vancheri, Sara Tomassetti, Carlo Agostini, Antonella Caminati, Marco Confalonieri, Rossana Della Porta, Paola Rogliani, Alfredo Sebastiani, Loreta Di Michele, Francesco Cinetto, Francesco Salton, Alice Biffi, Harari, Sergio, Caminati, Antonella, Confalonieri, Marco, Poletti, Venerino, Vancheri, Carlo, Pesci, Alberto, Paola, Rogliani, Luppi, Fabrizio, Agostini, Carlo, Rottoli, Paola, Sanduzzi Zamparelli, Alessandro, Sebastiani, Alfredo, Della Porta, Rossana, Salton, Francesco, Messore, Barbara, Tomassetti, Sara, Rosso, Roberta, Biffi, Alice, Puxeddu, Ermanno, Cerri, Stefania, Cinetto, Francesco, Refini, Rosa Metella, Bocchino, Marialuisa, Di Michele, Loreta, Specchia, Claudia, Albera, Carlo, Harari, S., Caminati, A., Confalonieri, M., Poletti, V., Vancheri, C., Pesci, A., Rogliani, Germana, Luppi, F., Agostini, C., Rottoli, P., Sanduzzi Zamparelli, A., Sebastiani, A., Della Porta, R., Salton, F., Messore, LUIGI GIULIO FRANCESCO, Tomassetti, S., Rosso, R., Biffi, A., Puxeddu, E., Cerri, S., Cinetto, F., Refini, R. M., Bocchino, M., Di Michele, L., Specchia, C., Albera, C., Harari, S, Caminati, A, Confalonieri, M, Poletti, V, Vancheri, C, Pesci, A, Rogliani, P, Luppi, F, Agostini, C, Rottoli, P, Sanduzzi Zamparelli, A, Sebastiani, A, Della Porta, R, Salton, F, Messore, B, Tomassetti, S, Rosso, R, Biffi, A, Puxeddu, E, Cerri, S, Cinetto, F, Refini, R, Bocchino, M, Di Michele, L, Specchia, C, and Albera, C
- Subjects
Male ,medicine.medical_treatment ,Settore MED/10 - Malattie dell'Apparato Respiratorio ,Anti-Inflammatory Agents ,Physiology ,Idiopathic pulmonary fibrosis ,0302 clinical medicine ,Primary outcome ,Risk Factors ,Immunology and Allergy ,Cumulative incidence ,030212 general & internal medicine ,Genetics (clinical) ,education.field_of_study ,Anti-Inflammatory Agents, Non-Steroidal ,Pirfenidone ,Middle Aged ,idiopathic pulmonary fibrosis ,Prognosis ,antifibrotic therapies ,mortality ,prognosis ,staging ,survival ,Pulmonary and Respiratory Medicine ,Treatment Outcome ,Female ,Non-Steroidal ,prognosi ,medicine.drug ,Lung Transplantation ,Aged ,Humans ,Idiopathic Pulmonary Fibrosis ,Mortality ,Pyridones ,Retrospective Studies ,Sex Factors ,Disease stages ,Population ,antifibrotic therapie ,03 medical and health sciences ,anti-fibrotic therapie ,anti-fibrotic therapies ,medicine ,Lung transplantation ,education ,MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO ,idiopathic pulmonary fibrosi ,business.industry ,Retrospective cohort study ,medicine.disease ,030228 respiratory system ,business - 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).
- Published
- 2017
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