title: Immune Inflammation and Disease Progression in Idiopathic Pulmonary Fibrosis.
abstract: The clinical course in idiopathic pulmonary fibrosis (IPF) is highly heterogeneous, with some patients having a slow progression and others an accelerated clinical and functional decline. This study aims to clinically characterize the type of progression in IPF and to investigate the pathological basis that might account for the observed differences in disease behavior. Clinical and functional data were analyzed in 73 IPF patients, followed long-time as candidates for lung transplantation. The forced vital capacity (FVC) change/year (< or ≥10% predicted) was used to define "slow" or "rapid" disease progression. Pathological abnormalities were quantified in the explanted lung of 41 out of 73 patients undergoing lung transplantation. At diagnosis, slow progressors (n = 48) showed longer duration of symptoms and lower FVC than rapid progressors (n = 25). Eleven slow and 3 rapid progressors developed an acute exacerbation (AE) during follow-up. Quantitative lung pathology showed a severe innate and adaptive inflammatory infiltrate in rapid progressors, markedly increased compared to slow progressors and similar to that observed in patients experiencing AE. The extent of inflammation was correlated with the yearly FVC decline (r = 0.52, p = 0.005). In conclusion an innate and adaptive inflammation appears to be a prominent feature in the lung of patients with IPF and could contribute to determining of the rate of disease progression.
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Balestro
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Fiorella
authors.initials: F
authors.lastname: Calabrese
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Graziella
authors.initials: G
authors.lastname: Turato
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Lunardi
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Erica
authors.initials: E
authors.lastname: Bazzan
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Marulli
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Davide
authors.initials: D
authors.lastname: Biondini
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Emanuela
authors.initials: E
authors.lastname: Rossi
authors.affiliation: Department of Clinical Medicine and Surgery, Federico II University, Napoli, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Sanduzzi
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Rea
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Rigobello
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Dario
authors.initials: D
authors.lastname: Gregori
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Simonetta
authors.initials: S
authors.lastname: Baraldo
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Spagnolo
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.|Respiratory Division Meakins-Christie Laboratories, McGill University, Montreal, Canada.
authors.forename: Manuel G
authors.initials: MG
authors.lastname: Cosio
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: Saetta
mesh_terms: D000328:Adult; D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000276:immunology / Q000473:pathology*; D007249:Inflammation / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged