Considerations for Lung Transplantation in Patients With Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive and ultimately fatal disease. Currently, there is no proven effective therapy, and lung transplantation is the only intervention associated with prolonged survival. However, outcomes after lung transplantation are worse for IPF than for other frequent indications such as chronic obstructive pulmonary disease and cystic fibrosis. IPF is one of the most frequent indications for lung transplantation. Available lungs are a scarce resource; therefore, the selection criteria are very important to optimize outcomes after lung transplantation. The optimal procedure (single vs. bilateral lung transplantation) in IPF is not clear and should be determined on a case-by-case basis. IPF patients are often critically ill with advanced respiratory failure requiring high levels of support before transplant, and there are several technical surgical challenges specific to IPF. IPF patients have worse survival after lung transplant, at least in part due to early mortality, but it remains a mainstay of therapy given the lack of effective alternatives.