LandmarkRCT

EXAFIP

Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomised, double-blind, placebo-controlled, phase 3 trial.

Lancet Respir Med, 2022 · Multicenter double-blind placebo-controlled trial

Population: 120 randomized, 119 treated/analyzed adults with acute/suspected AE-IPF; ventilated patients and active infection excluded
Intervention: Cyclophosphamide added to high-dose glucocorticoids
Comparator: Placebo plus glucocorticoids
Primary outcome: Three-month all-cause mortality
Result: 45% versus 31%; absolute difference 14.5 percentage points (95% CI −3.1 to 31.6), P=.10.

What the trial found

45% versus 31%; absolute difference 14.5 percentage points (95% CI −3.1 to 31.6), P=.10.

Clinical interpretation

No survival benefit and a concerning direction of effect support avoiding cyclophosphamide for AE-IPF. This does not establish a statistically significant harm estimate or apply to every autoimmune ILD.

Moderate certainty

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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