The Genetic Predisposition to Interstitial Lung Disease: Functional Relevance

Abstract

The approach to the genetic predisposition to diffuse lung disease (previously known as interstitial lung disease) poses a number of unique problems. The diffuse lung diseases comprise a large number of distinct clinical diseases. Of these, the idiopathic interstitial pneumonias are of particular importance because they include idiopathic pulmonary fibrosis (IPF), the most lethal form of diffuse lung disease, together with a number of diffuse lung disorders that have been (and continue to be) misdiagnosed as IPF.