Acute exacerbation of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive and ultimately fatal disease, the clinical course of which in individual patients is highly variable. Sudden deterioration of a patient's respiratory condition during a stable course is not uncommon. Many cases of uncertain etiology have been called acute exacerbation (AEx) of IPF, under the assumption of sudden acceleration of the underlying disease process. In recent years, several studies have reported the clinical significance of AEx-IPF. In 2007 Collard and colleagues created a Consensus Perspective, which proposed consensus definition and standard diagnostic criteria. This review primarily discusses studies performed after this Consensus Perspective.