An international working group report in the American Journal of Respiratory and Critical Care Medicine updates the 2016 definition of acute exacerbation of idiopathic pulmonary fibrosis and extends it to all fibrotic interstitial lung diseases.
The proposed definition is an acute respiratory event with increased symptoms or signs plus radiological or histological features of diffuse alveolar damage, with or without organising pneumonia, in a patient with known or newly diagnosed fibrotic ILD. Separately, the group introduces 'acute respiratory worsening' as an umbrella for acute deteriorations not due to diffuse alveolar damage — pulmonary oedema, bronchitis, pneumonia — while noting that severe pneumonia can itself trigger an exacerbation.
The distinction matters at the bedside, because the work-up and treatment of heart failure or infection differ from those of a true exacerbation, which still has no proven therapy. The report also sets out research priorities and how exacerbations should be counted in trials.
- In a patient with fibrotic ILD who deteriorates acutely, look for new bilateral ground-glass change on HRCT suggesting diffuse alveolar damage.
- Exclude heart failure, pulmonary embolism and infection, which fall under acute respiratory worsening.
- Severe pneumonia can trigger a true exacerbation; the two can coexist.
- Discuss prognosis early — exacerbations carry high mortality and no treatment is proven.
Why it matters
A shared definition across fibrotic ILDs makes diagnosis, trials and prognosis talks consistent.
Read the rest in the app
You have read your two free briefings this month. The app carries all 27 specialties, every morning, free — and this finding is waiting in it.
Scan to keep reading on your phone. No account needed to start.
Tomorrow morning, before your first patient
One edition a day for pulmonology, written by the desk, every claim tied to its paper. Six minutes.
Get the app — free