Lung transplantation in idiopathic pulmonary fibrosis

ABSTRACT

Introduction: Idiopathic Pulmonary Fibrosis (IPF) is a unique type of interstitial pneumonia in which progressive fibrosis can ultimately result in respiratory failure and death. The median survival of IPF remains dismal despite newer anti-fibrotic therapies. Lung transplantation is the only modality currently known to enhance survival for patients with IPF.

Areas covered: Since IPF is predominantly a disorder of the elderly, determination of the impact of co-morbidities is crucial for risk stratification of the individual patient. We review the potential effect of anti-fibrotic therapy in the pre and post-transplant period; and also discuss the factors that need to be considered in deciding between single lung and bilateral lung transplantation.

Expert commentary: Early referral to a transplant center is recommended for patients with IPF due to the high waiting list mortality. Evaluation of the transplant candidate should also be directed specifically at identifying co-morbidities that portend higher risk. While there has been a universal trend favoring bilateral lung transplantation over single lung transplantation for IPF, there are inherent pros and cons for both strategies and decisions should be individualized. Further studies are required to deduce the efficacy and safety of anti-fibrotic drugs in the immediate pre-and post-lung transplantation period.

KEYWORDS:

• IPF
• pulmonary fibrosis
• lung transplantation
• anti-fibrotic
• bilateral lung transplantation

Declaration of interest

R Girgid attended a speakers’ bureau for Genentech and Boehringer Ingelheim for Pirfenidone and Nintedanib. G Raghu consulted with Biogen, Boehringer Ingelheim, Celgene, Fibrogen, Gilead Sciences, Medlmmune, Promedior, Genentech/Roche, Sanfoi-Aventis, UCB, and Veracyte. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

The manuscript was not funded.