ConsensusCurrent

AE-fILD working group — Acute exacerbation in fibrotic interstitial lung disease: an international working group report.

International fibrotic ILD working group, 2026

Distinguish general acute respiratory worsening from acute exacerbation of fibrotic ILD. Treat reversible causes, individualize supportive ventilation and transplantation decisions, and use corticosteroids cautiously, especially in IPF. This is a working-group report, not a GRADE treatment guideline; no intervention has a proven universal survival benefit.

Expert opinionVery low certainty

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Clinical Context & Relevant Topics (1)

Disease Reference

Interstitial Lung Disease (ILD) in the ICU

Acute exacerbation and decompensated ILD: differential diagnosis, HRCT patterns, oxygen/NIV/HFNC, ventilation strategy, immunosuppression limits, and transplant decisions.

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