RT Journal Article
SR Electronic
T1 Clues in interstitial lung disease in children (chILD)
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA3143
DO 10.1183/13993003.congress-2016.PA3143
VO 48
IS suppl 60
A1 Snijders, Deborah
A1 de Col, Elena
A1 Barbato, Angelo
YR 2016
UL http://erj.ersjournals.com/content/48/suppl_60/PA3143.abstract
AB chILD comprises over 200 different rare lung conditions. The combined incidence of chILD is < 1 per 100,000 children. The rarity of the conditions, usually with non-specific presenting signs, is often associated with late and/or misdiagnosis. Outcomes are frequently poor. Clinically patients with chILD can be characterized by four manifestations: (1) respiratory symptoms; (2) usually non-specific chest radiological abnormalities; (3) possible decreased volumes on pulmonary function tests; (4) if biopsied, patterns of inflammation and fibrosis, +/-characteristic.Aim: We evaluate four patients with a complete clinical, radiological and histological picture according to the guidelines on chILD (Bush A et al thorax 2015;70:1078–1084)Results: Four patients with criteria for chILD syndrome have been evaluated by Clinical symptoms with evidence of hypoxaemia mostly during sleep and exercise; by Imaging with HRCT scans with the presence of ground glass opacities, interstitial thickening, and nodular formation; by histology with Inflammatory/Fibrosing constrictive bronchiolitis, lymphocytic interstitial pneumonitis (LIP), Cellular bronchiolitis and inflammatory pattern. Treatment was cased based but mostly by long term steroids and macrolide treatment, hydroxyclorochine and mycophenolate. Clinically patients remained sick improved over time.Conclusion: Comprising more than 200 different rare lung diseases and some with nonspecific alteration, accurate diagnosis as well as disease-correlated treatment is very difficult in chILD. The four patients showed different onset, different imaging and different histology and different treatments and different outcomes but all are under the same umbrella “chILD”.Funded by EU-FP7 n°[305653].