The histologic diagnosis of usual interstitial pneumonia of idiopathic pulmonary fibrosis. Where we are and where we need to go.

ABSTRACT

In the 50 years since its inception by Dr. Liebow, the diagnosis of usual interstitial pneumonia (UIP) by pathologists has changed significantly. This manuscript reviews the progressive history of the histologic diagnosis of UIP and summarizes the current state of histologic UIP and its relationship to the clinical syndrome idiopathic pulmonary fibrosis (IPF). Fibrotic lung disease mimics of UIP/IPF are reviewed and pearls for distinguishing these diseases from UIP/IPF are provided. Strategies for increasing the value of histologic assessment of biopsies in the setting of pulmonary fibrosis are also discussed.