Interstitial Lung Disease Unit, Royal Brompton and Harefield National Health Service (NHS) Foundation Trust, London, UK; Department of Cardiothoracic Pharmacology, National Heart and Lung Institute, Imperial College London, London, UK. Electronic address: [Email]
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis. Lung transplantation is the only intervention shown to increase life expectancy for patients with IPF, but it is associated with disease-specific challenges. In this Review, we discuss the importance of a proactive approach to the management of IPF comorbidities, including gastro-oesophageal reflux, pulmonary hypertension, coronary artery disease, and malignancy. With a donor pool too small to meet demand and unacceptably high mortality on transplant waiting lists, we discuss different systems used internationally to facilitate organ allocation. We explore the rapidly evolving landscape of transplantation for patients with IPF with regards to antifibrotic therapy, technological advances in extracorporeal life support, advances in understanding of the genetics of the disease, and the importance of a holistic multidisciplinary approach to care. Finally, we consider potential advances over the next decade that are envisaged to improve transplantation outcomes in patients with advanced IPF.