Evaluation of Different Diagnostic Modalities in Diagnosis of Egyptian Children with Interstitial Lung Disease

Abstract

hildhood interstitial lung diseases (ChILD) encompass a broad spectrum of rare pulmonary disorders. Diffuse parenchymal lung disease (DPLD) is a newly adopted more accurate term, as the disease may affect the alveoli, airways, blood vessels, lymphatics, and pleural spaces as well. In most developing Middle Eastern countries, ChILD is still underdiagnosed. Our objective was to describe and investigate cases diagnosed with chILD in a tertiary university hospital in Egypt. We analyzed data of consecutive subjects (<18 years of age) referred for further evaluation at the Children’s hospital, Ain Shams university. Diagnosis of chILD was made by a multidisciplinary team in accordance with the ERS criteria. The following information was obtained: demographic data, clinical characteristics, chest CT findings, laboratory studies, spirometry, BAL and histopathological findings. 22 subjects were enrolled over 24 months. Median age at Diagnosis was 7 years [range 3.5-14 years], and the sex ratio was 2.1 female/male. The most symptoms were dyspnea (100%), cough (90.9%) and recurrent pneumonias (77.3%). Digital clubbing (95.5%), tachypnea (90.9%), retractions (36.4%), tachycardia (68.2%) and failure to thrive (54.5%) were the most commonly observed signs. Clinical, Radiologic, laboratory and histopathology assessment 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), chILD related to systemic sclerosis (n=1), familial interstitial lung disease of unidentified aetiology (n=1).