Childhood interstitial lung disease: A systematic review.
OBJECTIVE
Childhood interstitial lung disease (chILD) is a group of rare chronic and complex disorders of variable pathology. There has been no systematic review of published chILD research. This study aimed to describe chILD classification systems, epidemiology, morbidity, treatments, outcomes, and the impact of chILD on families and the burden on health services.


METHODS
A systematic literature search for original studies on chILD was undertaken in the major biomedical databases to the end of December 2013. Epidemiological studies, case series and studies describing classification systems were included. Single case studies were excluded.


RESULTS
The search yielded 37 publications that met study criteria. Four different chILD classification systems have been proposed in the past decade. The incidence of chILD has been estimated at 0.13-16.2 cases/100,000 children/year. One to five new cases presented to individual hospitals each year. In developed countries, the median mortality was 13% (6-19%). Morbidity and outcomes were highly variable and not systematically reported. Corticosteroids and hydroxychloroquine were the most common treatments. The impact of chILD on families and the burden on health services has not been studied.


CONCLUSIONS
The heterogeneity of the chILD group of disorders, different determinations of what constitutes a chILD disorder and, a paucity of large epidemiological studies precludes consolidation of results across studies. Consensus on chILD classification is needed to support diagnosis and allow direct comparisons of research evidence. Active disease surveillance and international patient registries are required to advance understanding and management of chILD.
Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Sydney, Australia.|Australian Paediatric Surveillance Unit, Kids Research Institute, Westmead NSW 2145, Australia.
Neil J
NJ
Hime
Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Sydney, Australia.|Australian Paediatric Surveillance Unit, Kids Research Institute, Westmead NSW 2145, Australia.
Yvonne
Y
Zurynski
Department of Respiratory Medicine, The Children's Hospital at Westmead, Westmead NSW 2145, Australia.|Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney Clinical School, Sydney, Australia.
Dominic
D
Fitzgerald
Department of Respiratory Medicine, The Children's Hospital at Westmead, Westmead NSW 2145, Australia.|Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney Clinical School, Sydney, Australia.
Hiran
H
Selvadurai
Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Sydney, Australia.|Australian Paediatric Surveillance Unit, Kids Research Institute, Westmead NSW 2145, Australia.
Amy
A
Phu
Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Sydney, Australia.|Australian Paediatric Surveillance Unit, Kids Research Institute, Westmead NSW 2145, Australia.
Marie
M
Deverell
Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, Sydney, Australia.|Australian Paediatric Surveillance Unit, Kids Research Institute, Westmead NSW 2145, Australia.|The Children's Hospital at Westmead, Sydney, Australia.
Elizabeth J
EJ
Elliott
Department of Respiratory Medicine, Sydney Children's Hospital, Randwick NSW 2031, Australia.|Discipline of Paediatrics, School of Women's and Children's Health, UNSW Medicine, The University of New South Wales, Sydney, Australia.
Adam
A
Jaffe
D002648:Child; D017281:Cost of Illness; D005938:Glucocorticoids / Q000627:therapeutic use; D006296:Health Services / Q000706:statistics & numerical data; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D015994:Incidence; D017563:Lung Diseases, Interstitial / Q000145:classification* / Q000453:epidemiology* / Q000628:therapy
D005938:Glucocorticoids; D006886:Hydroxychloroquine
Childhood interstitial lung disease; Diffuse lung disease; Interstitial lung disease; chILD syndrome