- Design
- International working group report and literature review
- Population
- Patients with fibrotic ILD, including IPF and non-IPF subtypes
- Primary outcome
- Revised definition and diagnostic framework for acute exacerbation
- Effect
- Diffuse alveolar damage-based definition; 'acute respiratory worsening' for other causes
An international working group has updated the 2016 definition of acute exacerbation of idiopathic pulmonary fibrosis and extended it to every fibrotic interstitial lung disease, including hypersensitivity pneumonitis and connective tissue disease-associated ILD.
The proposed definition is an acute rise in respiratory symptoms or signs with imaging or histology consistent with diffuse alveolar damage, with or without organising pneumonia, in a patient with known or newly diagnosed fibrotic ILD. A separate term, acute respiratory worsening, covers deteriorations from other causes such as pulmonary oedema, bronchitis or pneumonia, though severe pneumonia can itself trigger an exacerbation.
The report summarises evidence rather than issuing treatment recommendations, and it notes there is still no proven effective therapy for acute exacerbation. Its practical value is diagnostic discipline: a structured search for infection, heart failure, pulmonary embolism and pneumothorax before labelling deterioration as an exacerbation, which carries a grave prognosis.
- Look for new bilateral ground-glass change on CT, beyond the fibrosis, when a fibrotic ILD patient deteriorates.
- Exclude heart failure, pulmonary embolism, pneumothorax and infection before diagnosing acute exacerbation.
- Record the event as acute respiratory worsening when another cause explains it.
- Discuss goals of care early, given the high mortality of true acute exacerbation.
Why it matters
Separating true exacerbation from treatable worsening changes both management and what to tell the family.
Don't overread it
This is a consensus definition, not new evidence on how to treat exacerbations.
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