ABSTRACT

I. Introduction Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonitis (IIP) and an increasingly frequent indication for lung transplantation (1). First described in 1872 by von Buhl as “cirrhosis of the lungs” (2), it is now recognized as a progressive, fibrotic process isolated to the lungs (3). Although we are learning much about its course, the primary cause remains unknown. No medical therapy has been shown to be beneficial, and recognizing this, most experts recommend no medical therapy outside of experimental trials. Even with the well-recognized risks and suboptimal outcomes, lung transplantation is the only therapy proven to improve and extend life for IPF patients (4). However, lung transplantation for IPF still remains a developing therapy with many important and controversial questions to be answered.