Pulmonary Radiological Manifestations of Humoral and Combined Immunodeficiencies in a Tertiary Pediatric Center

Mitra Khalili; Hossein Farzi; Sepideh Darougar; Fatemeh Hajijoo; Mehrnaz Mesdaghi; Mahboubeh Mansouri; Delara Babaie; Amir Hashemitari; Narges Eslami; Zahra Chavoshzadeh

doi:10.18502/ijaai.v20i6.8020

Mitra Khalili Department of Radiology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Hossein Farzi Department of Radiology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Sepideh Darougar Department of Pediatrics, Tehran Medical Sciences Branch, Islamic Azad University, Tehran, Iran
Fatemeh Hajijoo Department of Radiology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mehrnaz Mesdaghi Department of Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mahboubeh Mansouri Department of Immunology and Allergy, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Delara Babaie Department of Immunology and Allergy, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Amir Hashemitari East London NHS Foundation Trust, London, United Kingdom
Narges Eslami Department of Immunology and Allergy, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Zahra Chavoshzadeh Pediatric Infections Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/ijaai.v20i6.8020

Keywords: Lung; Pediatrics; Primary immunodeficiency diseases

Abstract

Respiratory diseases are considered as significant causes of morbidity and mortality in primary immunodeficiencies. This study aimed to reveal the radiologic patterns of thoracic involvement in these disorders.

A total of 58 patients, including 38 cases with combined cellular-humoral and 20 cases with humoral immunodeficiencies, were enrolled in this study. The “combined” group consisted of 12 cases with severe combined immunodeficiency (SCID) and 26 cases with combined immunodeficiency. The “humoral” group included seven patients with Hyper IgM syndrome (HIGMs), seven cases with common variable immunodeficiency (CVID), three patients with X-linked agammaglobulinemia, and three patients with other types of humoral primary immunodeficiencies (PIDs). The mean age of patients at the time of evaluation was 3.3±3.8 and 5.3±3.9 years in combined and humoral groups, respectively. The findings of chest X-rays and CT scans were interpreted and compared.

There was a significant difference for alveolar opacification between combined and humoral immunodeficiencies (58% vs. 30%). The bronchopneumonia-like pattern was detected as a significant finding in patients with SCID (42%) and HIGMs (43%). Atrophy of the thymus was detected significantly often in cases of SCID (67%). Two patients with CVID and lipopolysaccharide-responsive and beige-like anchor protein deficiency showed parenchymal changes of granulomatous lymphocytic interstitial lung disease. No significant difference was detected for bronchiectasis, bronchitis/bronchiolitis patterns, pleural effusion, and thoracic lymphadenopathy. lymphadenopathy.

Distinct subtypes of primary immunodeficiency may provoke differing and comparable radiological patterns of thoracic involvement; which can clue the clinician and radiologist to the diagnosis of the disease.