- Design
- International working group report and literature review
- Population
- Patients with idiopathic and non-idiopathic fibrotic interstitial lung disease
- Primary outcome
- Revised definition and framework for acute exacerbation and acute respiratory worsening
- Effect
- Acute exacerbation defined by new diffuse alveolar damage in any fibrotic ILD; acute respiratory worsening as a separate category
An international working group has updated the 2016 report on acute exacerbation of idiopathic pulmonary fibrosis and extended it to all fibrotic interstitial lung diseases.
The proposed definition of acute exacerbation is an acute respiratory event with increased symptoms or signs and imaging or histological features consistent with diffuse alveolar damage, with or without organising pneumonia, in a patient with known or newly diagnosed fibrotic ILD. A separate category, acute respiratory worsening, covers deterioration not due to diffuse alveolar damage, such as pulmonary oedema, bronchitis and pneumonia — though severe pneumonia can trigger an exacerbation.
The practical value is in the work-up. A patient with fibrotic ILD who becomes acutely breathless needs high-resolution CT looking for new bilateral ground glass, and a deliberate search for heart failure, infection and pulmonary embolism. The report notes that no treatment has been proven effective for acute exacerbation, which is why distinguishing it from treatable mimics matters.
- Arrange CT in a patient with fibrotic ILD and acute worsening, looking for new ground glass consistent with diffuse alveolar damage.
- Look actively for heart failure, infection and pulmonary embolism, which cause acute worsening without an exacerbation.
- Apply the same approach to non-IPF fibrotic ILD, not only IPF.
- Discuss prognosis and goals early; acute exacerbation carries high mortality and has no proven treatment.
Why it matters
It gives one definition across fibrotic ILDs and separates exacerbation from causes that have treatments.
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