title: Ten-Year Survival in Patients with Idiopathic Pulmonary Fibrosis After Lung Transplantation.
abstract: BACKGROUND
  Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal fibrosing lung disease with a median survival of approximately 3 years after diagnosis. The only medical option to improve survival in IPF is lung transplantation (LTX). The purpose of this study was to evaluate trajectory data of IPF patients listed for LTX and to investigate the survival after LTX.
  
  
  RESULTS
  Data were retrospectively collected from September 1989 until July 2011 of all IPF patients registered for LTX in the Netherlands. Patients were included after revision of the diagnosis based on the criteria set by the ATS/ERS/JRS/ALAT. Trajectory data, clinical data at time of screening, and donor data were collected. In total, 98 IPF patients were listed for LTX. During the waiting list period, 30 % of the patients died. Mean pulmonary artery pressure, 6-min walking distance, and the use of supplemental oxygen were significant predictors of mortality on the waiting list. Fifty-two patients received LTX with a median overall survival after transplantation of 10 years.
  
  
  CONCLUSIONS
  This study demonstrated a 10-year survival time after LTX in IPF. Furthermore, our study demonstrated a significantly better survival after bilateral LTX in IPF compared to single LTX although bilateral LTX patients were significantly younger.
authors.affiliation: Department of Pulmonology, Center of Interstitial Lung Diseases, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands. l.ten.klooster@antoniusziekenhuis.nl.
authors.forename: Liesbeth
authors.initials: L
authors.lastname: ten Klooster
authors.affiliation: Department of Pulmonary Medicine and Tuberculosis, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: George D
authors.initials: GD
authors.lastname: Nossent
authors.affiliation: Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Johanna M
authors.initials: JM
authors.lastname: Kwakkel-van Erp
authors.affiliation: Department of Pulmonology, Center of Interstitial Lung Diseases, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands.|Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Diana A
authors.initials: DA
authors.lastname: van Kessel
authors.affiliation: Department of Pulmonology, Center of Interstitial Lung Diseases, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands.|Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Erik J
authors.initials: EJ
authors.lastname: Oudijk
authors.affiliation: Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Ed A
authors.initials: EA
authors.lastname: van de Graaf
authors.affiliation: Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Bart
authors.initials: B
authors.lastname: Luijk
authors.affiliation: Department of Respiratory Medicine, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Rogier A
authors.initials: RA
authors.lastname: Hoek
authors.affiliation: Department of Respiratory Medicine, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Bernt
authors.initials: B
authors.lastname: van den Blink
authors.affiliation: Department of Respiratory Medicine, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Peter Th
authors.initials: PT
authors.lastname: van Hal
authors.affiliation: Department of Pulmonary Medicine and Tuberculosis, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Erik A
authors.initials: EA
authors.lastname: Verschuuren
authors.affiliation: Department of Pulmonary Medicine and Tuberculosis, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Wim
authors.initials: W
authors.lastname: van der Bij
authors.affiliation: Department of Pulmonology, Center of Interstitial Lung Diseases, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands.|Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Coline H
authors.initials: CH
authors.lastname: van Moorsel
authors.affiliation: Department of Pulmonology, Center of Interstitial Lung Diseases, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands.|Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Jan C
authors.initials: JC
authors.lastname: Grutters
mesh_terms: D015331:Cohort Studies; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology; D054990:Idiopathic Pulmonary Fibrosis / Q000453:epidemiology / Q000401:mortality / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D010102:Oxygen Inhalation Therapy / Q000706:statistics & numerical data; D011669:Pulmonary Wedge Pressure; D012189:Retrospective Studies; D015996:Survival Rate; D014850:Waiting Lists / Q000401:mortality
keywords: Interstitial lung diseases; Organ transplantation; Outcomes; Usual interstitial pneumonia