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Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Imaging findings

CT chest shows severe chronic interstitial lung disease characterized by a UIP pattern with lower-lobe reticulation, traction bronchiectasis, honeycombing, and airspace expansion with fibrosis, combined with upper-lobe emphysema (CPFE). Comparison with a scan from 4 weeks prior shows a rapid development of diffuse, bilateral ground-glass opacities, crazy paving, and consolidation, representing acute-on-chronic lung injury.

Key takeaways

Acute exacerbation of idiopathic pulmonary fibrosis (IPF) is defined by rapid clinical deterioration and new, bilateral ground-glass opacities or consolidation not explained by infection or heart failure. Radiographically, it manifests as acute organizing pneumonia or diffuse alveolar damage (DAD) superimposed on a background of chronic fibrosis, carrying a very poor prognosis.

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