Pneumologie 2023; 77(S 01): S53
DOI: 10.1055/s-0043-1760995
Abstracts

Nintedanib in children and adolescents with fibrosing interstitial lung disease: the InPedILD* trial** 

M Griese
1   Kinderklinik und Kinderpoliklinik; Haunersches Kinderspital; Hauner Children's Hospital, Ludwig Maximilians University, German Center for Lung Research (Dzl), Munich, Germany
,
R Deterding
2   Section of Pediatric Pulmonary and Sleep Medicine, Dept of Pediatrics, University of Colorado Denver, Denver, Co, Usa; The Children's Hospital Colorado, Aurora, Co, USA
,
L Young
3   Division of Pulmonary and Sleep Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pa, USA
,
E DeBoer
2   Section of Pediatric Pulmonary and Sleep Medicine, Dept of Pediatrics, University of Colorado Denver, Denver, Co, Usa; The Children's Hospital Colorado, Aurora, Co, USA
,
D Warburton
4   Children's Hospital Los Angeles, Los Angeles, Ca, Usa; Keck School of Medicine, University of Southern California, Los Angeles, Ca, USA
,
S Cunningham
5   Centre for Inflammation Research, University of Edinburgh, Edinburgh, UK
,
N Schwerk
6   Clinic for Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
,
K Flaherty
7   Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI, USA
,
K Brown
8   Department of Medicine, National Jewish Health, Denver, Co, USA
,
M Dumistracel
9   Boehringer Ingelheim Pharma GmbH & Co. Kg, Ingelheim am Rhein, Germany
,
E Erhardt
10   Boehringer Ingelheim Pharma GmbH & Co. Kg, Biberach, Germany
,
J Bertulis
10   Boehringer Ingelheim Pharma GmbH & Co. Kg, Biberach, Germany
,
M Gahlemann
11   Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland
,
S Stowasser
12   Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany
› Author Affiliations
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Background Nintedanib is approved for the treatment of pulmonary fibrosis in adults. There are no licensed treatments for fibrosing ILD in children and adolescents ([Abb. 1]).

Zoom Image
Abb. 1  

Aim The InPedILD trial was designed to determine the dosing and safety of nintedanib in children and adolescents with fibrosing ILD.

Methods Patients aged 6–17 years with fibrosing ILD on 2 HRCTs or on lung biopsy and HRCT (with the most recent HRCT in prior≤12 months), FVC≥25% predicted and clinically significant ILD were eligible. Patients were randomised 2:1 to receive nintedanib or placebo double-blind on top of standard of care for 24 weeks followed by open-label nintedanib. Dosing was based on weight-dependent allometric scaling. Co-primary endpoints are the area under the plasma concentration-time curve at steady state at weeks 2 and 26 and the proportion of patients with treatment-emergent adverse events during the double-blind period. FVC was measured at baseline, weeks 2, 6, 12, 24, and at regular visits thereafter.

Results The trial was conducted at 43 sites in 21 countries. Analysis of baseline characteristics from the 39 patients randomised is shown in the Table.

Conclusion The InPedILD trial provides insights into the risk benefit of nintedanib in children and adolescents with fibrosing ILD.



Publication History

Article published online:
09 March 2023

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