Extract
Interstitial lung diseases (ILDs) that present in childhood (chILD) are seen far less frequently than ILDs presenting in adults which themselves constitute rare disorders [1]. Histopathological [2, 3] and imaging [4] characterisation of chILD disease subtypes therefore lags behind adult ILDs. The field has also been constrained by comparisons with disease morphology in adults, despite the developmental differences in terms of growth and healing in the paediatric lung, which may alter disease patterns and distributions. The American Thoracic Society [5] and European [1] chILD management guidelines both specify a pivotal role for computed tomography (CT) imaging in the work-up of chILD patients to: 1) determine whether a chILD is present or not; and 2) where possible, to make a specific diagnosis of the underlying cause. For the second aim to be achieved, diagnostic reviews need to be reproducible between experts. Our study uniquely examined agreement between observers of varying experience in the CT evaluation of chILD to inform whether the current status of CT imaging and knowledge can be diagnostic of specific chILDs. We hypothesised that observer agreement for chILD groups and diagnoses would be limited. The study was not designed to relate CT agreement to final diagnosis. As a secondary analysis, we examined how CT interpretation differed between observers in children under and over 2 years of age.
Abstract
Making chILD diagnoses on CT is poorly reproducible, even amongst sub-specialists. CT might best improve diagnostic confidence in a multidisciplinary team setting when augmented with clinical, functional and haematological results. http://bit.ly/327jRCw
Footnotes
Author contributions: JJ, CMO, ASB, TS, TAW, AC, PG-P, PT, AM-G, PA, AD, HW, TJV, SC, AA, AUW, AGN, AR, AB were involved in either the acquisition, or analysis or interpretation of data for the study. JJ, CMO and AB were also involved in the conception and design of the study. All authors revised the work for important intellectual content and gave final approval for the version to be published. All authors agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Conflict of interest: J. Jacob reports personal fees from Boehringer Ingelheim (advisory board fees) and Roche, outside the submitted work.
Conflict of interest: C.M. Owens reports personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: A.S. Brody reports personal fees from Vertex, outside the submitted work.
Conflict of interest: T. Semple reports personal fees from Vertex, outside the submitted work.
Conflict of interest: T.A. Watson has nothing to disclose.
Conflict of interest: A. Calder has nothing to disclose.
Conflict of interest: P. Garcia-Peña has nothing to disclose.
Conflict of interest: P. Toma has nothing to disclose.
Conflict of interest: A. Devaraj reports personal fees from Boehringer Ingelheim, Roche, and GSK, outside the submitted work.
Conflict of interest: H. Walton has nothing to disclose.
Conflict of interest: A. Moreno-Galdó reports personal fees from Abbvie, Actelion, and Novartis, outside the submitted work.
Conflict of interest: P. Aurora has nothing to disclose.
Conflict of interest: A. Rice reports personal fees from AbbVie, outside the submitted work.
Conflict of interest: T.J. Vece has nothing to disclose.
Conflict of interest: S. Cunningham reports personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: A. Altmann has nothing to disclose.
Conflict of interest: A.U. Wells reports personal fees from Intermune (advisory board and speaker fees), Boehringer Inlgeheim (advisory board and speaker fees), Gilead (advisory board fees), MSD (advisory board fees), Roche (advisory board and speaker fees), Bayer (advisory board and speaker fees), and Chiesi (speaker fees), outside the submitted work.
Conflict of interest: A.G. Nicholson reports personal fees from Boehringer Ingelheim (advisory board fees), Roche, Medical Quantitative Image analysis, and Galapagos, outside the submitted work.
Conflict of interest: A. Bush has nothing to disclose.
Support statement: Joseph Jacob was supported by Wellcome Trust Clinical Research Career Development Fellowship 209553/Z/17/Z. Andrew Bush is an Emeritus NIHR Senior Investigator and is supported by chILD-EU (FP7, No: 305653) and the European Cooperation in Science and Technology COST A16125. Andre Altmann holds an MRC eMedLab Medical Bioinformatics Career Development Fellowship. This work was supported by the Medical Research Council (grant number MR/L016311/1). Funding information for this article has been deposited with the Crossref Funder Registry.
- Received April 20, 2019.
- Accepted June 6, 2019.
- Copyright ©ERS 2019
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