title: Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study.
abstract: The natural history of idiopathic pulmonary fibrosis (IPF) is not well defined and its clinical course is variable. We sought to investigate the survival and incidence of acute exacerbations (AEs) and their significant predictors in newly diagnosed patients. 70 patients newly diagnosed with IPF were prospectively followed for at least 3 yrs. Baseline evaluation included Medical Research Council dyspnoea score (MRCDS), 6-min walk test, pulmonary function tests, all of which were repeated at 6 months, and high-resolution computed tomography. A retrospective cohort of 68 patients was used for confirmation. Mean survival from the time of diagnosis was 30 months, with a 3-yr mortality of 46%. A Risk stratificatiOn ScorE (ROSE) based on MRCDS > 3, 6-min walking distance ≤ 72% predicted and composite physiologic index > 41 predicted 3-yr mortality with high specificity. 6-month progression of ROSE predicted rapid progression. 3-yr incidence of AE was 18.6%, mostly occurring in the first 18 months; risk factors for AE were concomitant emphysema and low diffusing coefficient of the lung for carbon monoxide. Results were confirmed in an independent cohort of patients. In newly diagnosed IPF, advanced disease at presentation, rapid progression and AEs are the determinants of 3-yr survival. The purpose of the multifactorial ROSE is to risk-stratify patients in order to predict survival and detect rapid disease progression.
authors.affiliation: Malattie Respiratorie, Policlinico Tor Vergata, Università di Roma Tor Vergata, Rome, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Mura
authors.forename: Maria A
authors.initials: MA
authors.lastname: Porretta
authors.forename: Elena
authors.initials: E
authors.lastname: Bargagli
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Sergiacomi
authors.forename: Maurizio
authors.initials: M
authors.lastname: Zompatori
authors.forename: Nicola
authors.initials: N
authors.lastname: Sverzellati
authors.forename: Amedeo
authors.initials: A
authors.lastname: Taglieri
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Mezzasalma
authors.forename: Paola
authors.initials: P
authors.lastname: Rottoli
authors.forename: Cesare
authors.initials: C
authors.lastname: Saltini
authors.forename: Paola
authors.initials: P
authors.lastname: Rogliani
mesh_terms: D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011658:Pulmonary Fibrosis / Q000401:mortality* / Q000503:physiopathology; D012372:ROC Curve; D012129:Respiratory Function Tests; D012307:Risk Factors; D016019:Survival Analysis