Clinical spectrum of chronic interstitial lung disease in children

J Pediatr. 1992 Dec;121(6):867-72. doi: 10.1016/s0022-3476(05)80330-0.


To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortality rates.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Biopsy
  • Child
  • Child, Preschool
  • Chronic Disease
  • Colorado / epidemiology
  • Follow-Up Studies
  • Humans
  • Infant
  • Lung / diagnostic imaging
  • Lung / pathology
  • Prevalence
  • Pulmonary Fibrosis / diagnosis*
  • Pulmonary Fibrosis / epidemiology
  • Pulmonary Fibrosis / therapy
  • Radiography
  • Respiratory Function Tests
  • Retrospective Studies
  • Treatment Outcome