Idiopathic interstitial pneumonitis in children: a national survey in the United Kingdom and Ireland.
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.
Dinwiddie
R
R
Respiratory Unit, Great Ormond Street Hospital for Children, London, UK. R.Dinwiddie@ich.ucl.ac.uk
Sharief
N
N
Crawford
O
O
D000293:Adolescent; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D006306:Health Surveys; D006801:Humans; D007223:Infant; D007494:Ireland / Q000453:epidemiology; D017563:Lung Diseases, Interstitial / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy; D008297:Male; D016896:Treatment Outcome; D006113:United Kingdom / Q000453:epidemiology