Fibrotic lung diseases in children

Pediatr Pulmonol. 2024 May;59(5):1165-1174. doi: 10.1002/ppul.26905. Epub 2024 Feb 14.

Abstract

In children, pulmonary fibrosis (PF) is an extremely unusual entity that can be observed in some types of interstitial lung disease (ILD). Defining whether ILD is accompanied by PF is important for targeted therapy. Algorithm for the diagnosis of PF in children is not clearly established. Besides, the clinical, radiological, and histological definitions commonly used to diagnose particularly the cases of idiopathic PF in adult patients, is not applicable to pediatric cases. However, a few studies conducted in children offer good exemplary diagnostic approach to fibrosing ILD. Thorax high resonance computed tomography and/or lung biopsy scanning can provide valuable information about PF. Another issue that has not been clearly established is when to start antifibrotic treatment in pediatric patients with PF. The objective of this current review is to provide a comprehensive overview of pediatric PF by drawing upon adult research, particularly focusing on the areas of uncertainty.

Keywords: antifibrotic; children; interstitial lung disease; nintedanib; pirfenidone; pulmonary fibrosis.

Publication types

  • Review

MeSH terms

  • Biopsy
  • Child
  • Humans
  • Lung / diagnostic imaging
  • Lung / pathology
  • Lung Diseases, Interstitial* / diagnosis
  • Lung Diseases, Interstitial* / diagnostic imaging
  • Pulmonary Fibrosis / diagnostic imaging
  • Tomography, X-Ray Computed*

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