Acute exacerbation of interstitial lung disease
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 .
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.
Epidemiology
The annual incidence of AE-IPF has been reported at around 5-15% on one publication and is considered is less common in mild disease.
Pathology
Alveolar damage is considered the predominant feature of AE-ILD which manifests histopathologically as diffuse alveolar damage.
Radiographic features
CT
May manifest as rather diffuse, bilateral ground-glass opacification on a background of more established preexisting areas of pulmonary fibrosis.
Treatment and prognosis
It is often associated with a poor prognosis and high mortality . Empirical high-dose corticosteroid steroid therapy has been used but without proven benefit.
See also
