Original Article
  

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

Pulmonary Manifestations of Immunodeficiencies

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.
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.

1. Picard C, Bobby Gaspar H, Al-Herz W, Bousfiha A, Casanova J-L, Chatila T, et al. International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity. J Clin Immunol. 2018;38(1):96–128.
2. Jesenak M, Banovcin P, Jesenakova B, Babusikova E. Pulmonary manifestations of primary immunodeficiency disorders in children. Front Pediatr. 2014;2(1):77-9.
3. Madkaikar M, Mishra A, Ghosh K. Diagnostic approach to primary immunodeficiency disorders. Indian Pediatr. 2013;50(6):579–86.
4. Hampson FA, Chandra A, Screaton NJ, Condliffe A, Kumararatne DS, Exley AR, et al. Respiratory disease in common variable immunodeficiency and other primary immunodeficiency disorders. Clin Radiol. 2012;67(6):587–95.
5. Bierry G, Boileau J, Barnig C, Gasser B, Korganow AS, Buy X, et al. Thoracic manifestations of primary humoral immunodeficiency: a comprehensive review. Radiographics. 2009;29(7):1909–20.
6. Cereser L, Girometti R, d’Angelo P, De Carli M, De Pellegrin A, Zuiani C. Humoral primary immunodeficiency diseases: clinical overview and chest high-resolution computed tomography (HRCT) features in the adult population. Clin Radiol. 2017;72(7):534–42.
7. Winningham PJ, Martínez-Jiménez S, Rosado-de-Christenson ML, Betancourt SL, Restrepo CS, Eraso A. Bronchiolitis: A Practical Approach for the General Radiologist-Erratum. Radiographics. 2017;37(5):1607-9.
8. Yazdani R, Abolhassani H, Asgardoon M, Shaghaghi M, Modaresi M, Azizi G, et al. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders. J Investig Allergol Clin Immunol. 2017;27(4):213–24.
9. Dosanjh A. Chronic pediatric pulmonary disease and primary humoral antibody based immune disease. Respir Med. 2011;105(4):511–4.
10. Pandit C, Hsu P, van Asperen P, Mehr S. Respiratory manifestations and management in children with Common Variable Immunodeficiency. Paediatr Respir Rev. 2016;19(8):56–61.
11. Babaie D, Atashpar S, Chavoshzadeh Z, Armin S, Mesdaghi M, Fahimzad A, et al. Surveillance of Primary Immunodeficiency Disorders in Mofid Children’s Hospital: A 10-year retrospective experience. Arch Pediatr Infect Dis [Internet]. 2017;In Press(In Press):e61642. Available from: https://sites.kowsarpub.com/apid/articles/61642.html
12. Bondioni MP, Duse M, Plebani A, Soresina A, Notarangelo LD, Berlucchi M, et al. Pulmonary and sinusal changes in 45 patients with primary immunodeficiencies: computed tomography evaluation. J Comput Assist Tomogr. 2007;31(4):620–8.
13. Mogica Martínez MD, García Lara S, Silva Vera R, Montaño Velázquez B, Cruz Mérida A, Santibáñez Bustamante J, et al. [Neumopathies in patients with primary immunodeficiencies in treatment with intravenous gammaglobulin]. Rev Alerg Mex. 2007;54(1):14–9.
14. Membrila-Mondragón J, Staines-Boone AT, Sánchez-Sánchez LM, Ruiz-Pedraza MD. [Pulmonary complications in pediatric patients with primary immunodeficiency]. Gac Med Mex. 2015;151(2):157–63.
15. Goussault H, Salvator H, Catherinot E, Chabi M-L, Tcherakian C, Chabrol A, et al. Primary immunodeficiency-related bronchiectasis in adults: comparison with bronchiectasis of other etiologies in a French reference center. Respir Res. 2019;20.
16. Nambu A, Ozawa K, Kobayashi N, Tago M. Imaging of community-acquired pneumonia: Roles of imaging examinations, imaging diagnosis of specific pathogens and discrimination from noninfectious diseases. World J Radiol. 2014;6(10):779–93.
17. Szczawińska-Popłonyk A, Jończyk-Potoczna K, Ossowska L, Bręborowicz A, Bartkowska-Śniatkowska A, Wachowiak J. Cytomegalovirus pneumonia as the first manifestation of severe combined immunodeficiency. Cent J Immunol. 2014;39(3):392–5.
18. Fernández Pérez ER. Granulomatous lymphocytic interstitial lung disease. Immunol Allergy Clin North Am. 2012;32(4):621–32.
19. Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, et al. British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract. 2017;5(4):938–45.
20. Ohshimo S, Guzman J, Costabel U, Bonella F. Differential diagnosis of granulomatous lung disease: clues and pitfalls: Number 4 in the Series “Pathology for the clinician” Edited by Peter Dorfmüller and Alberto Cavazza. Eur Respir Rev. 2017;26(145).
21. Rao N, Mackinnon AC, Routes JM. Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency--histologic and immunohistochemical analyses of 16 cases. Hum Pathol. 2015;46(9):1306–14.
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IssueVol 20 No 6 (2021) QRcode
SectionOriginal Article(s)
DOI https://doi.org/10.18502/ijaai.v20i6.8020
Keywords
Lung Pediatrics Primary immunodeficiency diseases
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How to Cite
1.
Khalili M, Farzi H, Darougar S, Hajijoo F, Mesdaghi M, Mansouri M, Babaie D, Hashemitari A, Eslami N, Chavoshzadeh Z. Pulmonary Radiological Manifestations of Humoral and Combined Immunodeficiencies in a Tertiary Pediatric Center. Iran J Allergy Asthma Immunol. 2021;20(6):693-699.