• Liesbeth ten Klooster, George D Nossent, Johanna M Kwakkel-van Erp, Diana A van Kessel, Erik J Oudijk, Ed A van de Graaf, Bart Luijk, Rogier A Hoek, Bernt van den Blink, Peter Th van Hal, Erik A Verschuuren, Wim van der Bij, Coline H van Moorsel, and Jan C Grutters.
• Department of Pulmonology, Center of Interstitial Lung Diseases, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, The Netherlands. l.ten.klooster@antoniusziekenhuis.nl.
• Lung. 2015 Dec 1; 193 (6): 919-26.

IntroductionIdiopathic 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 ResultsData 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.ConclusionsThis 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.

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