Mortality in idiopathic pulmonary fibrosis (IPF) on the waiting list for lung transplantation in the Netherlands

Background: Idiopathic Pulmonary Fibrosis (IPF) is a progressive disease with a poor prognosis of 2.5 to 5 years. IPF is not responsive to medicinal treatment and lung transplantation is the only therapeutic option to prolong life. Objective: The aim of this study was to examine waiting list mortality of IPF in the Netherlands. Methods: Data were retrospectively collected from September 1989 till June 2010 of all IPF patients registered for lung transplantation in the Netherlands. Patients were included after revision of the diagnosis based on the IPF criteria set by the ATS/ERS. Clinical data and lung function measurements were collected at the time of screening. Results: 167 IPF patients were referred for lung transplantation. After evaluation for contraindications and screening, 90 patients were listed for lung transplantation. During the waiting list period 33.3% of IPF patients (n=30) died compared to 13.8% in Cystic Fibrosis (CF) (p = 0.0018) and 16.3% in Chronic Obstructive Pulmonary Disease (COPD) (p = 0.003). Analysis of lung function showed a mean FVC%-predicted of 51.1% (SD 19.0) and mean DLco%-predicted of 27.1% (SD 9.4) at time of screening. Five patients were taken off the list due to new comorbidities and deterioration of physical condition, 51 were transplanted and 4 IPF patients were still on the waiting list. Conclusions: This study revealed a significantly higher waiting list mortality for IPF compared to COPD and CF. DLco%-predicted at time of screening was considerably lower than international guidelines for lung transplantation. This indicates that timing of referral of IPF for lung transplantation can be improved.