Pathogenesis of IPF: Summary

The Course of IPF Progression, Small

Adapted from: Wolters PJ, Collard HR, Jones KD. Annu Rev Pathol Mech Dis. 2014;9:157-179.

The earliest descriptions of IPF date back more than 140 years, and through the course of the past several decades, there have been dramatic advances in characterization of the pathogenic mechanisms that underlie IPF. At present, the best evidence suggests that genetic and environmental factors contribute to IPF susceptibility, and in the setting of injurious stimuli, lead to failure of appropriate repair mechanisms, culminating instead with fibroblast recruitment and proliferation, myofibroblast differentiation, and extracellular matrix remodeling that over time manifests as progressive lung fibrosis.

Content contributed by:
Jonathan A. Kropski, MD
Vanderbilt University