Interstitial Lung Disease and Lung Transplantation

Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias and carries the worst prognosis. Currently the best treatment option is lung transplantation. Historically patients with IPF had poor outcomes following referral for lung transplant due to high waiting-list mortality. The introduction of the lung allocation score in the United States in 2005 has reduced 1 year waiting-list mortality from 21% to 11% and also led to IPF becoming the most common diagnosis for lung transplantation. Although prioritizing all patients with IPF on the waiting list has led to a dramatic decline in waiting-list mortality, further improvements may be made by prioritizing which patients with IPF should be transplanted, with an emphasis on 6-minute walk testing, biomarkers, and the presence of pulmonary hypertension rather than traditional pulmonary function tests. The choice of surgical procedure and the prediction and management of posttransplant complications may also influence long-term outcomes.