Abstract

Acute exacerbations (AE) of interstitial pneumonia are characterised by a rapid decline in respiratory function and oxygenation, and the appearance of new radiological abnormalities: ground-glass opacification/consolidation superimposed on pre-existing patterns of interstitial pneumonia, typically during the preceding month in patients with chronic fibrosing interstitial pneumonia. While AE is associated with a high mortality rate, survivors of AE are severely impaired and are potential candidates for consideration of lung transplantation. First reported in idiopathic pulmonary fibrosis (IPF) [1], AEs have also been reported in other idiopathic interstitial pneumonias (IIPs), such as nonspecific interstitial pneumonia (NSIP), fibrotic interstitial lung disease (ILD) that includes fibrotic hypersensitivity pneumonitis (FHP), and connective tissue disease-related ILDs.