Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disorder and ultimately fatal disease. Acute exacerbation of IPF (AE-IPF) is defined as a rapid deterioration with new radiographic opacities in patients with IPF with no identifiable cause such as infection, pulmonary embolism, or heart failure. Reported incidence of this disease entity is highly variable and the etiology is unknown. Research into the pathobiology of this process has implicated possible causes including microaspirations and occult viral infection. AE-IPF manifests histopathologically as diffuse alveolar damage and can have features of organizing pneumonia in the later phase. High-resolution computed tomography of the chest demonstrates new ground-glass opacities and can have prognostic value based on the pattern and extent of opacities. The mortality of this condition is very high and currently there is no proven effective treatment other than lung transplantation. AE-IPF is a major cause of morbidity and mortality in patients with IPF and further research is needed to elucidate the pathobiology and develop effective treatments.