Spectrum of childhood interstitial and diffuse lung diseases at a tertiary hospital in Egypt

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.