Idiopathic interstitial pneumonitis in children: a national survey in the United Kingdom and Ireland

Pediatr Pulmonol. 2002 Jul;34(1):23-9. doi: 10.1002/ppul.10125.


Interstitial lung disease (ILD) of unknown etiology in immunocompetent patients is rare in children. A national survey was carried out in the United Kingdom and Ireland over a 3-year period in order to identify prevalence, age distribution, histopathology, natural history of the illness, and response to current treatment.Forty-six cases were identified, including 29 males and 17 females. Seventy-six percent presented in the first year of life. Nine (16%) occurred within four families. Conventional treatment with pulsed methylprednisolone, prednisolone, or hydroxychloroquine, singly or in combination, resulted in an excellent response in 65% of cases. Seven children died (15%). The recurrence risk for further children within the same family to develop ILD is estimated to be approximately 10%. The prevalence rate of this condition in the United Kingdom and Ireland during the period of study for children aged 0-16 years is estimated to be 3.6 cases/million.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Age Distribution
  • Child
  • Child, Preschool
  • Female
  • Health Surveys*
  • Humans
  • Infant
  • Ireland / epidemiology
  • Lung Diseases, Interstitial / epidemiology*
  • Lung Diseases, Interstitial / physiopathology
  • Lung Diseases, Interstitial / therapy
  • Male
  • Treatment Outcome
  • United Kingdom / epidemiology