Lung transplantation in idiopathic pulmonary fibrosis

Expert Rev Respir Med. 2018 May;12(5):375-385. doi: 10.1080/17476348.2018.1462704. Epub 2018 Apr 20.

Abstract

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; anti-fibrotic; bilateral lung transplantation; lung transplantation; pulmonary fibrosis.

Publication types

  • Review

MeSH terms

  • Aged
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Idiopathic Pulmonary Fibrosis / surgery
  • Lung Transplantation*
  • Waiting Lists