Lung transplantation in idiopathic pulmonary fibrosis: old & new concepts

Taking into account the poor prognosis and limited treatment options, including lung transplantation (LTX) for only a selected number of patients, and the unpredictable clinical course of idiopathic pulmonary fibrosis (IPF) and often delay in referral of patients to a tertiary centre for interstitial lung diseases, the aim of this thesis was to evaluate the results of LTX in IPF patients in the Netherlands and to identify factors that might help to improve the outcome of LTX in IPF patients in the future.

The first part of this thesis provides a historical overview of all IPF patients referred for LTX since the start of the transplant program in the Netherlands and evaluates the waiting list mortality of IPF patients. This thesis revealed that one-third of the IPF patients died awaiting LTX. Furthermore, evaluating of the survival outcomes after LTX demonstrated a 10-year survival time after LTX in IPF patients in the Netherlands.

Another aim was to identify new prognostic biomarkers in patient’s blood or lung lavage fluids that might help to optimize scoring models like the Lung Allocation Score (LAS) and to guide clinicians in the complex matter of referral and listing for LTX. This thesis demonstrated the potential of IgA in serum as a prognostic biomarker in patients with IPF.

Furthermore, this thesis focuses on different genetic mutations identified in familial and sporadic IIPs. We aimed to investigate patients with pulmonary fibrosis and genetic mutations involved in surfactant homeostasis, telomere maintenance and lamellar bodies in order to define the clinical relevance of the genetic contribution to the disease. This thesis investigated the prevalence, clinical characteristics and prognosis in these patients and discuss the potential consequences for LTX.