Patients with acute exacerbations of interstitial lung disease (ILD) who are managed using a standardized protocol and also have idiopathic pulmonary fibrosis (IPF) have a higher risk of death or transplant at 1-year compared to those without a diagnosis of IPF, researchers found.

Moreover, an improvement on chest computed tomography (CT) 7 days after corticosteroid pulse is associated with better survival. These findings are reported in a new study by Canadian researchers that appeared in the journal Lung.

To assess the feasibility and outcomes of a standardized management protocol for acute exacerbations of ILD, the team of researchers, led by Jolene H Fisher, MD, conducted a retrospective cohort study of 89 patients admitted to a hospital between January 2015 and August 2019 due to acute exacerbation of ILD. The patients were managed with a standardized protocol. This included a chest CT at diagnosis, pulse corticosteroid treatment, and a follow-up CT 7 days after corticosteroid pulse.

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The 1-year transplant-free survival was 20.2% and the overall survival was 51.7%. 

“A diagnosis of IPF was associated with a higher risk of death or transplant at 1-year versus a non-IPF diagnosis,” the researchers found.

Read more about IPF prognosis

Although there were no significant associations with 7-day CT improvement, on adjusted analysis, the researchers found that CT improvement was associated with higher transplant-free survival and a lower risk of hospital mortality.

“To our knowledge, this is the first study suggesting a potential benefit of a short interval follow-up CT scan for prognostication of [acute exacerbations of] ILD,” the authors of the study wrote. They added that further prospective studies are needed to determine the optimal management of these patients, especially in terms of the role of corticosteroid therapy.

Currently, guidelines for the treatment of acute exacerbations of IPF give a weak recommendation for corticosteroid treatment.


Adams CJ, Chohan K, Rozenberg D, et al. Feasibility and outcomes of a standardized management protocol for acute exacerbation of interstitial lung disease. Lung. Published online August 4, 2021. doi:10.1007/s00408-021-00463-5