Acute exacerbation of interstitial lung disease

Last revised by Yuranga Weerakkody on 3 Mar 2023

Acute exacerbation of interstitial lung disease (AE-ILD), which sometimes also encompasses acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF), is an acute, clinically significant deterioration which develops within less than 1 month without an obvious clinical precipitant such as fluid overload, infection, left heart failure, or pulmonary embolism 2,7.

At the time of initial writing (2018) the majority of data regarding AE-ILD comes from the idiopathic pulmonary fibrosis (IPF) population and is extrapolated to other forms of ILD. 

The annual incidence of AE-IPF has been reported at around 5-15% on one publication 6, and is considered is less common in mild disease.

Alveolar damage is considered the predominant feature of AE-ILD which manifests histopathologically as diffuse alveolar damage.

May manifest as rather diffuse, bilateral ground-glass opacification on a background of more established preexisting areas of pulmonary fibrosis.

It is often associated with a poor prognosis and high mortality 3. Empirical high-dose corticosteroid steroid therapy has been used but without proven benefit 5.

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