{"doi":"10.1183/13993003.01512-2022","title":"Nintedanib in children and adolescents with fibrosing interstitial lung diseases","abstract":"Background Childhood interstitial lung disease (ILD) comprises a spectrum of rare ILDs affecting infants, children and adolescents. Nintedanib is a licensed treatment for pulmonary fibrosis in adults. The primary objectives of the InPedILD trial were to determine the dose-exposure and safety of nintedanib in children and adolescents with fibrosing ILD. Methods Patients aged 6–17 years with fibrosing ILD on high-resolution computed tomography and clinically significant disease were randomised 2:1 to receive nintedanib or placebo for 24 weeks and then open-label nintedanib. Dosing was based on weight-dependent allometric scaling. Co-primary end-points were the area under the plasma concentration–time curve at steady state (AUC τ,ss ) at weeks 2 and 26 and the proportion of patients with treatment-emergent adverse events at week 24. Results 26 patients received nintedanib and 13 patients received placebo. The geometric mean (geometric coefficient of variation) AUC τ,ss for nintedanib was 175 µg·h·L −1 (85.1%) in patients aged 6–11 years and 167 µg·h·L −1 (83.6%) in patients aged 12–17 years. In the double-blind period, adverse events were reported in 84.6% of patients in each treatment group. Two patients discontinued nintedanib due to adverse events. Diarrhoea was reported in 38.5% and 15.4% of the nintedanib and placebo groups, respectively. Adjusted mean± se changes in percentage predicted forced vital capacity at week 24 were 0.3±1.3% in the nintedanib group and −0.9±1.8% in the placebo group. Conclusions In children and adolescents with fibrosing ILD, a weight-based dosing regimen resulted in exposure to nintedanib similar to adults and an acceptable safety profile. These data provide a scientific basis for the use of nintedanib in this patient population.","journal":"European Respiratory Journal","year":2022,"id":235402,"datarank":2.4844209839828375,"base_score":4.406719247264253,"endowment":4.406719247264253,"self_citation_contribution":0.6610078870896381,"citation_network_contribution":1.8234130968931996,"self_endowment_contribution":0.6610078870896381,"citer_contribution":1.8234130968931996,"corpus_percentile":null,"corpus_rank":null,"citation_count":81,"citer_count":58,"citers_with_citation_signal":41,"citers_with_endowment":41,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9565,"is_data_producer":true,"deposit_databanks":{"ClinicalTrials.gov":["NCT04093024"],"EudraCT":["2018-004530-14"]},"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2022-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":289420,"name":"Lisa R. Young","orcid":"0000-0003-3297-8532","position":1,"is_corresponding":false},{"id":777369,"name":"Emily M. DeBoer","orcid":"0000-0002-0924-9342","position":2,"is_corresponding":false},{"id":564361,"name":"David Warburton","orcid":"0000-0002-4605-1298","position":3,"is_corresponding":false},{"id":853289,"name":"Steve Cunningham","orcid":"0000-0001-7342-251X","position":4,"is_corresponding":false},{"id":760889,"name":"Nicolaus Schwerk","orcid":"0000-0002-5496-7749","position":5,"is_corresponding":false},{"id":278241,"name":"Kevin R. Flaherty","orcid":"0000-0001-7686-0291","position":6,"is_corresponding":false},{"id":264771,"name":"Kevin K. Brown","orcid":"0000-0002-9209-3261","position":7,"is_corresponding":false},{"id":854041,"name":"Mihaela Dumistracel","orcid":null,"position":8,"is_corresponding":false},{"id":853290,"name":"Elvira Erhardt","orcid":"0000-0003-3885-1479","position":9,"is_corresponding":false},{"id":853291,"name":"Julia Bertulis","orcid":"0000-0002-4863-6003","position":10,"is_corresponding":false},{"id":854042,"name":"Martina Gahlemann","orcid":null,"position":11,"is_corresponding":false},{"id":853292,"name":"Susanne Stowasser","orcid":"0009-0008-6013-0225","position":12,"is_corresponding":false},{"id":106364,"name":"Matthias Griese","orcid":"0000-0003-0113-912X","position":13,"is_corresponding":false},{"id":853288,"name":"Robin R. Deterding","orcid":"0000-0001-6890-3466","position":0,"is_corresponding":true}],"reference_count":29,"raw_metadata":null,"created_at":"2026-07-19T00:21:48.116752Z","pmid":"36041751","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}