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 a situation as 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-IPF 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.
Treatment and prognosis
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.
- 1. Azadeh N, Moua T, Baqir M, Ryu JH. Treatment of acute exacerbations of interstitial lung disease. (2018) Expert review of respiratory medicine. 12 (4): 309-313. doi:10.1080/17476348.2018.1446831 - Pubmed
- 2. Leuschner G, Behr J. Acute Exacerbation in Interstitial Lung Disease. (2017) Frontiers in medicine. 4: 176. doi:10.3389/fmed.2017.00176 - Pubmed
- 3. Kolb M, Bondue B, Pesci A, Miyazaki Y, Song JW, Bhatt NY, Huggins JT, Oldham JM, Padilla ML, Roman J, Shapera S. Acute exacerbations of progressive-fibrosing interstitial lung diseases. (2018) European respiratory review : an official journal of the European Respiratory Society. doi:10.1183/16000617.0071-2018 - Pubmed
- 4. Luppi F, Cerri S, Taddei S, Ferrara G, Cottin V. Acute exacerbation of idiopathic pulmonary fibrosis: a clinical review. (2015) Internal and emergency medicine. 10 (4): 401-11. doi:10.1007/s11739-015-1204-x - Pubmed
- 5. Antoniou KM, Wells AU. Acute exacerbations of idiopathic pulmonary fibrosis. (2013) Respiration; international review of thoracic diseases. 86 (4): 265-74. doi:10.1159/000355485 - Pubmed
- 6. Ryerson CJ, Cottin V, Brown KK, Collard HR. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. (2015) The European respiratory journal. 46 (2): 512-20. doi:10.1183/13993003.00419-2015 - Pubmed
- 7. Hyzy R, Huang S, Myers J, Flaherty K, Martinez F. Acute exacerbation of idiopathic pulmonary fibrosis. (2007) Chest. 132 (5): 1652-8. doi:10.1378/chest.07-0299 - Pubmed