Diagnostic workup of childhood interstitial lung disease

Eur Respir Rev. 2023 Feb 21;32(167):220188. doi: 10.1183/16000617.0188-2022. Print 2023 Mar 31.

Abstract

Childhood interstitial lung diseases (chILDs) are rare and heterogeneous diseases with significant morbidity and mortality. An accurate and quick aetiological diagnosis may contribute to better management and personalised treatment. On behalf of the European Respiratory Society Clinical Research Collaboration for chILD (ERS CRC chILD-EU), this review summarises the roles of the general paediatrician, paediatric pulmonologists and expert centres in the complex diagnostic workup. Each patient's aetiological chILD diagnosis must be reached without prolonged delays in a stepwise approach from medical history, signs, symptoms, clinical tests and imaging, to advanced genetic analysis and specialised procedures including bronchoalveolar lavage and biopsy, if necessary. Finally, as medical progress is fast, the need to revisit a diagnosis of "undefined chILD" is stressed.

Publication types

  • Review

MeSH terms

  • Biopsy / adverse effects
  • Bronchoalveolar Lavage / adverse effects
  • Child
  • Diagnostic Imaging
  • Humans
  • Lung / pathology
  • Lung Diseases, Interstitial* / therapy
  • Morbidity