title: European protocols for the diagnosis and initial treatment of interstitial lung disease in children.
abstract: Interstitial lung disease in children (chILD) is rare, and most centres will only see a few cases/year. There are numerous possible underlying diagnoses, with specific and non-specific treatment possibilities. The chILD-EU collaboration has brought together centres from across Europe to advance understanding of these considerations, and as part of this process, has created standard operating procedures and protocols for the investigation of chILD. Where established consensus documents exist already, for example, for the performance of bronchoalveolar lavage and processing of lung biopsies, these have been adopted. This manuscript reports our proposals for a staged investigation of chILD, starting from when the condition is suspected to defining the diagnosis, using pathways dependent on the clinical condition and the degree of illness of the child. These include the performance of genetic testing, echocardiography, high-resolution CT, bronchoscopy when appropriate and the definitive investigation of lung biopsy, in order to establish a precise diagnosis. Since no randomised controlled trials of treatment have ever been performed, we also report a Delphi consensus process to try to harmonise treatment protocols such as the use of intravenous and oral corticosteroids, and add-on therapies such as hydroxychloroquine and azithromycin. The aim is not to dictate to clinicians when a therapeutic trial should be performed, but to offer the possibility to collaborators of having a unified approach when a decision to treat has been made.
authors.affiliation: Imperial College, National Heart and Lung Institute, Royal Brompton Harefield NHS Foundation Trust, London, UK.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bush
authors.affiliation: NHS Lothian and University of Edinburgh, Edinburgh, UK.
authors.forename: Steve
authors.initials: S
authors.lastname: Cunningham
authors.affiliation: Pediatric Pulmonary Department, Hôpital Universitaire Necker Enfants Malades, Paris, France Université Paris Descartes, Paris, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: de Blic
authors.affiliation: Department of Women's and Children's Health, University of Padova, Padua, Italy.
authors.forename: Angelo
authors.initials: A
authors.lastname: Barbato
authors.affiliation: Hôpital Armand-Trousseau, Pneumologie pédiatrique, Centre National de Référence des Maladies Respiratoires Rares, Paris, France.
authors.forename: Annick
authors.initials: A
authors.lastname: Clement
authors.affiliation: Faculté de Médecine, Centre Intercommunal de Créteil, Service de Pédiatrie, INSERM, U955, Université Paris-Est, Créteil, France.
authors.forename: Ralph
authors.initials: R
authors.lastname: Epaud
authors.affiliation: Children's Hospital of Ludwig, Maximilians University, Munich, Germany.
authors.forename: Meike
authors.initials: M
authors.lastname: Hengst
authors.affiliation: Faculty of Medicine, Department of Pediatric Pulmonology, Hacettepe University, Ankara, Turkey.
authors.forename: Nural
authors.initials: N
authors.lastname: Kiper
authors.affiliation: Imperial College & Royal Brompton Harefield NHS Foundation Trust, London, UK.
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Nicholson
authors.affiliation: Department of Pediatrics, Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Wetzke
authors.affiliation: Department of Women's and Children's Health, University of Padova, Padua, Italy.
authors.forename: Deborah
authors.initials: D
authors.lastname: Snijders
authors.affiliation: Department of Pediatric Pulmonology and Pediatric Lung Transplantation, Hannover Medical School, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hannover, Germany.
authors.forename: Nicolaus
authors.initials: N
authors.lastname: Schwerk
authors.affiliation: Lung Research Group, Children's Hospital of Ludwig, Maximilians University, Munich, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Griese
mesh_terms: D018893:Bronchoalveolar Lavage / Q000379:methods*; D001999:Bronchoscopy / Q000379:methods*; D002648:Child; D002985:Clinical Protocols; D003937:Diagnosis, Differential; D019468:Disease Management; D005060:Europe / Q000453:epidemiology; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D009017:Morbidity; D014057:Tomography, X-Ray Computed / Q000379:methods*
keywords: ABCA3; GM-CSF Receptor; Hypersensitivity pneumonitis; Idiopathic pulmonary fibrosis; NEHI; PIG; Surfactant protein; TTF-1