title: Mortality on the Waiting List for Lung Transplantation in Patients with Idiopathic Pulmonary Fibrosis: A Single-Centre Experience.
abstract: OBJECTIVE
  Lung transplantation (LTX) is nowadays accepted as a treatment option for selected patients with end-stage pulmonary disease. Idiopathic pulmonary fibrosis (IPF) is characterized by the radiological and histologic appearance of usual interstitial pneumonia. It is associated with a poor prognosis, and LTX is considered an effective treatment to significantly modify the natural history of this disease. The aim of the present study was to analyse mortality during the waiting list in IPF patients at a single institution.
  
  
  METHODS
  A retrospective analysis on IPF patients (n = 90) referred to our Lung Transplant Program in the period 2001-2014 was performed focusing on patients' characteristics and associated risk factors.
  
  
  RESULTS
  Diagnosis of IPF was associated with high mortality on the waiting list with respect to other diagnosis (p < 0.05). No differences in demographic, clinical, radiological data and time spent on the waiting list were observed between IPF patients who underwent to LTX or lost on the waiting list. Patients who died showed significant higher levels of pCO2 and needed higher flows of O2-therapy on effort (p < 0.05). Pulmonary function tests failed to predict mortality and no other medical conditions were associated with survival.
  
  
  CONCLUSIONS
  Patients newly diagnosed with IPF, especially in small to medium lung transplant volume centres and in Countries where a long waiting list is expected, should be immediately referred to transplantation, delay results in increased mortality. Early identification of IPF patients with a rapid progressive phenotype is strongly needed.
authors.affiliation: Respiratory Diseases and Lung Transplantation Unit, Internal and Specialist Medicine Department, Azienda Ospedaliera Universitaria Senese (AOUS), Viale Bracci n° 16, 2nd Floor, 3rd Building, 53100, Siena, Italy. david.btt@gmail.com.
authors.forename: David
authors.initials: D
authors.lastname: Bennett
authors.affiliation: Respiratory Diseases and Lung Transplantation Unit, Internal and Specialist Medicine Department, Azienda Ospedaliera Universitaria Senese (AOUS), Viale Bracci n° 16, 2nd Floor, 3rd Building, 53100, Siena, Italy.
authors.forename: Antonella
authors.initials: A
authors.lastname: Fossi
authors.affiliation: Respiratory Diseases and Lung Transplantation Unit, Internal and Specialist Medicine Department, Azienda Ospedaliera Universitaria Senese (AOUS), Viale Bracci n° 16, 2nd Floor, 3rd Building, 53100, Siena, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Bargagli
authors.affiliation: Respiratory Diseases and Lung Transplantation Unit, Internal and Specialist Medicine Department, Azienda Ospedaliera Universitaria Senese (AOUS), Viale Bracci n° 16, 2nd Floor, 3rd Building, 53100, Siena, Italy.
authors.forename: Rosa Metella
authors.initials: RM
authors.lastname: Refini
authors.affiliation: Respiratory Diseases and Lung Transplantation Unit, Internal and Specialist Medicine Department, Azienda Ospedaliera Universitaria Senese (AOUS), Viale Bracci n° 16, 2nd Floor, 3rd Building, 53100, Siena, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Pieroni
authors.affiliation: Thoracic Surgery Unit, Cardio-Thoracic-Vascular Department, Azienda Ospedaliera Universitaria Senese (AOUS), Siena, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Luzzi
authors.affiliation: Thoracic Surgery Unit, Cardio-Thoracic-Vascular Department, Azienda Ospedaliera Universitaria Senese (AOUS), Siena, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Ghiribelli
authors.affiliation: Thoracic Surgery Unit, Cardio-Thoracic-Vascular Department, Azienda Ospedaliera Universitaria Senese (AOUS), Siena, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Paladini
authors.affiliation: Thoracic Surgery Unit, Cardio-Thoracic-Vascular Department, Azienda Ospedaliera Universitaria Senese (AOUS), Siena, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Voltolini
authors.affiliation: Respiratory Diseases and Lung Transplantation Unit, Internal and Specialist Medicine Department, Azienda Ospedaliera Universitaria Senese (AOUS), Viale Bracci n° 16, 2nd Floor, 3rd Building, 53100, Siena, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Rottoli
mesh_terms: D000328:Adult; D000368:Aged; D002245:Carbon Dioxide / Q000097:blood; D003550:Cystic Fibrosis / Q000401:mortality / Q000601:surgery; D005080:Exercise Test; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000401:mortality* / Q000628:therapy; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D010100:Oxygen / Q000008:administration & dosage; D010102:Oxygen Inhalation Therapy; D010313:Partial Pressure; D029424:Pulmonary Disease, Chronic Obstructive / Q000401:mortality / Q000601:surgery; D012189:Retrospective Studies; D013997:Time Factors; D014850:Waiting Lists / Q000401:mortality*; D016138:Walking / Q000502:physiology
chemical_list: D002245:Carbon Dioxide; D010100:Oxygen
keywords: Idiopathic pulmonary fibrosis; Lung transplant; Mortality; Usual interstitial pneumonia; Waiting list