PT - JOURNAL ARTICLE AU - Salma G. Abdelhady AU - Eman M. Fouda AU - Malak A. Shaheen AU - Faten A. Ghazal AU - Ahmed M. Mostafa AU - Ahmed M. Osman AU - Andrew G. Nicholson AU - Heba M. Hamza TI - Spectrum of childhood interstitial and diffuse lung diseases at a tertiary hospital in Egypt AID - 10.1183/23120541.00880-2020 DP - 2021 Jan 01 TA - ERJ Open Research PG - 00880-2020 4099 - http://openres.ersjournals.com/content/early/2021/03/04/23120541.00880-2020.short 4100 - http://openres.ersjournals.com/content/early/2021/03/04/23120541.00880-2020.full AB - Background Childhood interstitial and diffuse lung diseases (chILD) encompass a broad spectrum of rare pulmonary disorders. In most developing middle eastern countries, chILD is still underdiagnosed. Objective: To describe and investigate patients diagnosed with chILD in a tertiary university hospital in Egypt.Methods We analyzed data of consecutive subjects (<18 years) referred for further evaluation at the Children's Hospital, Ain Shams University. Diagnosis of chILD was made in accordance with the chILD-EU criteria. The following information was obtained: demographic data, clinical characteristics, chest CT findings, laboratory studies, spirometry, BAL and histopathology findings.Results 22 subjects were enrolled over 24 months. Median age at diagnosis was 7 years (range 3.5–14 years). The most common manifestations were dyspnea (100%), cough (90.9%), clubbing (95.5%) and tachypnea (90.9%). Systematic evaluation led to the following diagnoses: hypersensitivity pneumonitis (n=3), idiopathic interstitial pneumonias (n=4), chILD related to chronic granulomatous disease (n=3), chILD related to small airway disease (n=3), postinfectious chILD (n=2), Langerhans cell histiocytosis (n=2), Idiopathic pulmonary hemosiderosis (n=2), granulomatous lymphocytic interstitial lung disease (n=1), systemic sclerosis (n=1), familial interstitial lung disease (n=1). Among the subjects who completed the diagnostic evaluation (n=19), treatment was changed in 13 (68.4%) subjects.Conclusion Systematic evaluation and multidisciplinary peer review of chILD patients at our tertiary hospital led to changes in management in 68% of the patients. This study also highlights the need for an Egyptian chILD network with genetic testing, as well as the value of collaborating with international groups in improving health care for children with chILD.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Abdelhady has nothing to disclose.Conflict of interest: Dr. Fouda has nothing to disclose.Conflict of interest: Dr. Shaheen has nothing to disclose.Conflict of interest: Dr. Ghazal has nothing to disclose.Conflict of interest: Dr. Mostafa has nothing to disclose.Conflict of interest: Dr. Osman has nothing to disclose.Conflict of interest: A.G. Nicholson reports personal fees for consultancy on idiopathic pulmonary fibrosis (IPF) trials and education from Boehringer Ingelheim, and personal fees for consultancy on IPF trials from Medical Quantitative Image Analysis and Galapagos, outside the submitted work.Conflict of interest: Dr. Hamza has nothing to disclose.