Lisa R. Young,1 Kevin K. Brown,2 Matthias Griese,3 Gail Deutsch,4,5 David Warburton,6,7 Emily DeBoer,8,9 Steven Cunningham,10 Annick Clement,11 Nicolaus Schwerk,12 Kevin Flaherty,13 Florian Voss,14 Ulrike Schmid,14 Rozsa Schlenker-Herceg,15 Daniela Verri,16 Susanne Stowasser,17 Emmanuelle Clerisme-Beaty,17 Mihaela Dumistracel,17 Marilisa Schiwek,17 Kay Tetzlaff,17
Robin Deterding8,9

1Division of Pulmonary Medicine, The Children’s Hospital of Philadelphia, Philadelphia, PA; 2Department of Medicine, National Jewish Health, Denver, CO; 3Hauner Children’s Hospital, Ludwig Maximilian University, Munich, Germany; 4Department of Pathology, University of Washington School of Medicine, Seattle, WA; 5Seattle Children’s Hospital, Seattle, WA; 6Children’s Hospital Los Angeles, Los Angeles, CA; 7Keck School of Medicine, University of Southern California, Los Angeles, CA; 8Section of Pediatric Pulmonary and Sleep Medicine, Department of Pediatrics, University of Colorado Denver, Denver, CO; 9The Children’s Hospital Colorado, Aurora, CO; 10Centre for Inflammation Research, University of Edinburgh, Edinburgh, United Kingdom; 11Pediatric Pulmonary Department, Trousseau Hospital, AP-HP Sorbonne University, Paris, France; 12Clinic for Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; 13Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI; 14Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, Germany; 15Boehringer Ingelheim Pharmaceuticals Inc., Ridgefield, CT; 16Boehringer Ingelheim Italia S.p.A, Milan, Italy; 17Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany

 

Childhood interstitial lung disease (chILD)

  • >200 heterogeneous respiratory disorders
  •  Conditions associated with fibrosing chILD include:
    • Connective tissue disease‑related ILD
    • Radiation- and drug-induced fibrosis
    • Surfactant dysfunction disorders
    • Other

There are currently no approved treatments for fibrosing ILD in children

Nintedanib, a tyrosine kinase inhibitor with a distinct inhibitory spectrum,1 has been shown to reduce the rate of decline in FVC

  • In adult patients with IPF2 and SSc-ILD3
  • In patients with chronic fibrosing ILDs with a progressive phenotype, independent of fibrotic HRCT pattern4
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