International management platform for children's interstitial lung disease (chILD-EU)

Thorax. 2018 Mar;73(3):231-239. doi: 10.1136/thoraxjnl-2017-210519. Epub 2017 Oct 22.


Background: Children's interstitial lung diseases (chILD) cover many rare entities, frequently not diagnosed or studied in detail. There is a great need for specialised advice and for internationally agreed subclassification of entities collected in a register.Our objective was to implement an international management platform with independent multidisciplinary review of cases at presentation for long-term follow-up and to test if this would allow for more accurate diagnosis. Also, quality and reproducibility of a diagnostic subclassification system were assessed using a collection of 25 complex chILD cases.

Methods: A web-based chILD management platform with a registry and biobank was successfully designed and implemented.

Results: Over a 3-year period, 575 patients were included for observation spanning a wide spectrum of chILD. In 346 patients, multidisciplinary reviews were completed by teams at five international sites (Munich 51%, London 12%, Hannover 31%, Ankara 1% and Paris 5%). In 13%, the diagnosis reached by the referring team was not confirmed by peer review. Among these, the diagnosis initially given was wrong (27%), imprecise (50%) or significant information was added (23%).The ability of nine expert clinicians to subcategorise the final diagnosis into the chILD-EU register classification had an overall exact inter-rater agreement of 59% on first assessment and after training, 64%. Only 10% of the 'wrong' answers resulted in allocation to an incorrect category. Subcategorisation proved useful but training is needed for optimal implementation.

Conclusions: We have shown that chILD-EU has generated a platform to help the clinical assessment of chILD.

Trial registration number: Results, NCT02852928.

Keywords: paediatric interstitial lung disease; paediatric lung disaese; rare lung diseases.

Publication types

  • Clinical Trial
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Lung Diseases, Interstitial / diagnosis*
  • Male
  • Registries
  • Reproducibility of Results
  • Young Adult

Associated data