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European protocols for the diagnosis and initial treatment of interstitial lung disease in children
  1. Andrew Bush1,
  2. Steve Cunningham2,
  3. Jacques de Blic3,4,
  4. Angelo Barbato5,
  5. Annick Clement6,
  6. Ralph Epaud7,
  7. Meike Hengst8,
  8. Nural Kiper9,
  9. Andrew G Nicholson10,
  10. Martin Wetzke11,
  11. Deborah Snijders5,
  12. Nicolaus Schwerk12,
  13. Matthias Griese13
  14. on behalf of the chILD-EU collaboration
  1. 1Imperial College, National Heart and Lung Institute, Royal Brompton Harefield NHS Foundation Trust, London, UK
  2. 2NHS Lothian and University of Edinburgh, Edinburgh, UK
  3. 3Pediatric Pulmonary Department, Hôpital Universitaire Necker Enfants Malades, Paris, France
  4. 4Université Paris Descartes, Paris, France
  5. 5Department of Women's and Children's Health, University of Padova, Padua, Italy
  6. 6Hôpital Armand-Trousseau, Pneumologie pédiatrique, Centre National de Référence des Maladies Respiratoires Rares, Paris, France
  7. 7Faculté de Médecine, Centre Intercommunal de Créteil, Service de Pédiatrie, INSERM, U955, Université Paris-Est, Créteil, France
  8. 8Children's Hospital of Ludwig, Maximilians University, Munich, Germany
  9. 9Faculty of Medicine, Department of Pediatric Pulmonology, Hacettepe University, Ankara, Turkey
  10. 10Imperial College & Royal Brompton Harefield NHS Foundation Trust, London, UK
  11. 11Department of Pediatrics, Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
  12. 12Department of Pediatric Pulmonology and Pediatric Lung Transplantation, Hannover Medical School, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hannover, Germany
  13. 13Lung Research Group, Children's Hospital of Ludwig, Maximilians University, Munich, Germany
  1. Correspondence to Professor Andrew Bush, Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; a.bush{at}imperial.ac.uk

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.

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