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Review

The Burden of Non-Infectious Organ-Specific Immunopathology in Pediatric Common Variable Immunodeficiency

by
Aleksandra Szczawińska-Popłonyk
1,*,
Julia Bekalarska
2,
Kacper Jęch
2,
Nadia Knobloch
2,
Oliwia Łukasik
2,
Aleksandra Ossowska
2,
Jędrzej Ruducha
2 and
Zuzanna Wysocka
2
1
Department of Pediatric Pneumonology, Allergy and Clinical Immunology, Institute of Pediatrics, Poznan University of Medical Sciences, 61-701 Poznań, Poland
2
Student Scientific Society, Poznan University of Medical Sciences, 61-701 Poznań, Poland
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2025, 26(6), 2653; https://doi.org/10.3390/ijms26062653
Submission received: 15 January 2025 / Revised: 1 March 2025 / Accepted: 11 March 2025 / Published: 15 March 2025
(This article belongs to the Special Issue Molecular Advances in Inborn Errors of Immunity)
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Abstract

The pediatric common variable immunodeficiency (CVID) is the most frequent symptomatic antibody production defect characterized by infectious and non-infectious autoimmune, inflammatory, and lymphoproliferative complications. The background for CVID-related organ-specific immunopathology is associated with immune dysregulation and immunophenotypic biomarkers with expansion of CD21low B cells, and dysfunctional memory B cell, follicular T cell, and regulatory T cell compartments. The ever-increasing progress in immunogenetics shows the heterogeneity of genetic background for CVID related to the complexity of clinical phenotypes. Multiple systemic modulatory pathways are determined by variants in such genes as TACI or TNFRSF13B gene encoding for BAFF-R, CTLA-4, LRBA, NFKB1 and NFKB2, and PIK3CD or PIK3R1. The organ-specific immunopathology encompasses a spectrum of disorders associated with immune dysregulation, such as granulomatous interstitial lung disease, hepatocellular nodular regenerative hyperplasia, enteropathy, neuropathy, endocrinopathies, and dermatoses. This review is aimed to define and delineate the organ-specific immunopathology in pediatric CVID. It is also conducted to gather data facilitating a better understanding of complex and heterogeneous immunophenotypes in the context of immune dysregulation mechanisms and genetic background determining manifestations of the disease and implicating personalized targeted therapies with biological agents.
Keywords: autoimmunity; common variable immunodeficiency; enteropathy; granulomatous lymphocytic interstitial lung disease; immune dysregulation; nodular regenerative hyperplasia autoimmunity; common variable immunodeficiency; enteropathy; granulomatous lymphocytic interstitial lung disease; immune dysregulation; nodular regenerative hyperplasia

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MDPI and ACS Style

Szczawińska-Popłonyk, A.; Bekalarska, J.; Jęch, K.; Knobloch, N.; Łukasik, O.; Ossowska, A.; Ruducha, J.; Wysocka, Z. The Burden of Non-Infectious Organ-Specific Immunopathology in Pediatric Common Variable Immunodeficiency. Int. J. Mol. Sci. 2025, 26, 2653. https://doi.org/10.3390/ijms26062653

AMA Style

Szczawińska-Popłonyk A, Bekalarska J, Jęch K, Knobloch N, Łukasik O, Ossowska A, Ruducha J, Wysocka Z. The Burden of Non-Infectious Organ-Specific Immunopathology in Pediatric Common Variable Immunodeficiency. International Journal of Molecular Sciences. 2025; 26(6):2653. https://doi.org/10.3390/ijms26062653

Chicago/Turabian Style

Szczawińska-Popłonyk, Aleksandra, Julia Bekalarska, Kacper Jęch, Nadia Knobloch, Oliwia Łukasik, Aleksandra Ossowska, Jędrzej Ruducha, and Zuzanna Wysocka. 2025. "The Burden of Non-Infectious Organ-Specific Immunopathology in Pediatric Common Variable Immunodeficiency" International Journal of Molecular Sciences 26, no. 6: 2653. https://doi.org/10.3390/ijms26062653

APA Style

Szczawińska-Popłonyk, A., Bekalarska, J., Jęch, K., Knobloch, N., Łukasik, O., Ossowska, A., Ruducha, J., & Wysocka, Z. (2025). The Burden of Non-Infectious Organ-Specific Immunopathology in Pediatric Common Variable Immunodeficiency. International Journal of Molecular Sciences, 26(6), 2653. https://doi.org/10.3390/ijms26062653

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