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Idiopathic interstitial pneumonitis in children: A national survey in the United Kingdom and Ireland
Author(s) -
Dinwiddie R.,
Sharief N.,
Crawford O.
Publication year - 2002
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.10125
Subject(s) - medicine , pediatrics , hydroxychloroquine , etiology , prednisolone , interstitial lung disease , methylprednisolone , natural history , family history , surgery , disease , lung , covid-19 , infectious disease (medical specialty)
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. Pediatr Pulmonol. 2002; 34:23–29. © 2002 Wiley‐Liss, Inc.