Methods

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Inclusion and exclusion criteria

Key inclusion criteria

  • Aged 6–17 years
  • Evidence of fibrosing ILD
  • FVC ≥25% predicted
  • Clinically significant disease:
    • Fan score ≥3, or
    • Evidence of clinical progression over time:
      • 5–10% relative decline in FVC % predicted + worsening symptoms
      • ≥10% relative decline in FVC % predicted
      • Increased fibrosis on HRCT
      • Other measures, e.g. increased oxygen requirement, decreased diffusion capacity

Key exclusion criteria

  • Underlying chronic liver disease
  • Significant PAH
  • Cardiovascular disease (defined by protocol)
  • Bleeding risk

Evidence of fibrosing ILD, as confirmed by central review

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aCo-existing cystic abnormalities or ground-glass opacity are acceptable; however, co-existing multifocal, non-fibrotic, non-dependent consolidations (e.g. organizing pneumonia, infection) will not be permitted.
bWith or without ground-glass opacification
HRCT, high-resolution computed tomography; ILD, interstitial lung disease; NSIP, nonspecific interstitial pneumonia; UIP, usual interstitial pneumonia.

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