RADIOLOGICAL PATTERNS OF PULMONARY INVOLVEMENT IN HUMORAL AND COMBINED IMMUNODEFICIENCIES IN PEDIATRICS

Abstract

Backgrounds: Primary immunodeficiencies (PID) are a heterogeneous, wide-spectrum group of 354 distinct disorders with a variable spectrum of clinical manifestations. Respiratory disease is a significant cause of morbidity and mortality in these disorders. The most common characteristic radiographic and CT abnormalities recognized are noninfectious airway disorders, infections, chronic lung diseases, chronic inflammatory conditions (granulomatosis, interstitial pneumonias) and neoplasms. The aim of this study is to delineate and categorize the radiologic patterns of thoracic involvement in various subtypes of PID. Patients and Methods: A total of 58 patients with diagnosis of humoral, cellular or combined primary immunodeficiencies referred to Mofid Children’s Hospital, Shahid Beheshti University of medical sciences, Tehran, Iran, were enrolled in the study from 2011 to 2017. Their imaging findings were interpreted and compared according to their specific immunodeficiencies. Results: There was a significant difference for alveolar opacification in combined immunodeficiencies in comparison with humoral immunodeficiencies (58% vs. 30%, p value: 0.04). Bronchopneumonia- like pattern has been detected as a significant finding (p value: 0.01) in patients with severe combined immunodeficiency (42%) and Hyper IgM syndrome (43%); compared with other subtypes of immunodeficiency. Two patients with CVID and associated lipopolysacharide responsive beige- like anchor protein (LRBA) deficiency showed parenchymal changes suggestive of granulomatous lymphocytic interstitial lung disease (GLILD). Atrophy of thymus was significantly associated with severe combined immunodeficiency (67%, p value: 0.001). No significant difference was reported comparing bronchiectasis, bronchitis/ bronchiolitis pattern, pleural effusion and thoracic lymphadenopathy in patients with either humoral or cellular immunodeficiencies. Conclusions: Primary immunodeficiency syndromes may provoke radiologic patterns of thoracic involvement, which may lead the clinician and radiologist to potential diagnosis of disease, and contribute to differentiation of subtypes of the disorder.