Ten-Year Survival in Patients with Idiopathic Pulmonary Fibrosis After Lung Transplantation

• Published in: Lung Vol. 193; no. 6; pp. 919 - 926
• Main Authors: ten Klooster, Liesbeth, Nossent, George D., Kwakkel-van Erp, Johanna M., van Kessel, Diana A., Oudijk, Erik J., van de Graaf, Ed A., Luijk, Bart, Hoek, Rogier A., van den Blink, Bernt, van Hal, Peter Th, Verschuuren, Erik A., van der Bij, Wim, van Moorsel, Coline H., Grutters, Jan C.
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.12.2015; Springer Nature B.V
• Subjects: Cohort Studies; Exercise Test; Female; Humans; Hypertension, Pulmonary - epidemiology; Idiopathic Pulmonary Fibrosis - epidemiology; Idiopathic Pulmonary Fibrosis - mortality; Idiopathic Pulmonary Fibrosis - surgery; Lung Transplantation; Lungs; Male; Medicine; Medicine & Public Health; Middle Aged; Mortality; Netherlands - epidemiology; Oxygen Inhalation Therapy - utilization; Pneumology/Respiratory System; Pulmonary fibrosis; Pulmonary Wedge Pressure; Retrospective Studies; Survival Rate; Transplants & implants; Waiting Lists - mortality; Netherlands; Usual interstitial pneumonia; Organ transplantation; Interstitial lung diseases; Outcomes
• ISSN: 0341-2040; 1432-1750
• DOI: 10.1007/s00408-015-9794-7

Introduction 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. Methods and 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.