Interstitial lung disease in infants: new classification system, imaging technique, clinical presentation and imaging findings

Pediatr Radiol. 2013 Jan;43(1):3-13; quiz p.128-9. doi: 10.1007/s00247-012-2524-x. Epub 2012 Nov 15.


Interstitial lung disease (ILD) is defined as a rare, heterogeneous group of parenchymal lung conditions that develop primarily because of underlying developmental or genetic disorders. Affected infants typically present with clinical syndromes characterized by dyspnea, tachypnea, crackles and hypoxemia. Until recently, the understanding of ILD in infants has been limited largely owing to a lack of evidence-based information of underlying pathogenesis, natural history, imaging findings and histopathological features. However, ILD in infants is now better understood and managed because of (1) advances in imaging methods that result in rapid and accurate detection, (2) improved thoracoscopic techniques for lung biopsy, (3) a consensus regarding the pathological criteria for these particular lung conditions and (4) a new classification system based on the underlying etiology of ILD. This article reviews the new classification system, imaging technique, clinical presentation and imaging findings of ILD in infants. Specialized knowledge of this new classification system in conjunction with recognition of characteristic imaging findings of ILD in infants has great potential for early and accurate diagnosis, which in turn can lead to optimal patient management.

Publication types

  • Review

MeSH terms

  • Glycogen Storage Disease / complications
  • Humans
  • Hyperplasia
  • Hypertension, Pulmonary / complications
  • Infant
  • Infant, Newborn
  • Lung / abnormalities
  • Lung / diagnostic imaging*
  • Lung / growth & development
  • Lung Diseases, Interstitial / classification*
  • Lung Diseases, Interstitial / diagnosis*
  • Multidetector Computed Tomography
  • Mutation
  • Neuroendocrine Cells / pathology
  • Patient Positioning
  • Pulmonary Alveoli / abnormalities
  • Pulmonary Alveoli / diagnostic imaging
  • Pulmonary Surfactant-Associated Proteins / genetics
  • Radiation Dosage
  • Respiration
  • Respiratory Distress Syndrome, Newborn / etiology
  • Tomography, X-Ray Computed


  • Pulmonary Surfactant-Associated Proteins