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World J Methodol. Dec 20, 2024; 14(4): 96380
Published online Dec 20, 2024. doi: 10.5662/wjm.v14.i4.96380
Relation between dysbiosis and inborn errors of immunity
Öner Özdemir
Öner Özdemir, Department of Pediatric Allergy and Immunology, Sakarya University, Medical Faculty, Adapazarı 54100, Sakarya, Türkiye
Author contributions: Özdemir Ö has done everything.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Öner Özdemir, MD, Full Professor, Department of Pediatric Allergy and Immunology, Sakarya University Research and Training Hospital, Adnan Menderes Cad, Adapazarı 54100, Sakarya, Türkiye. ozdemir_oner@hotmail.com
Received: May 5, 2024
Revised: May 27, 2024
Accepted: June 14, 2024
Published online: December 20, 2024
Processing time: 81 Days and 19.9 Hours
Abstract

Inborn errors of immunity (IEI) disorders, formerly primary immune deficiency diseases, are a heterogeneous group of disorders with variable hereditary transitions, clinical manifestations, complications and varying disease severity. Many of the clinical symptoms, signs and complications in IEI patients can be attributed to inflammatory and immune dysregulatory processes due to loss of microbial diversity (dysbiosis). For example, in common variable immunodeficiency patients, the diversity of bacteria, but not fungi, in the gut microbiota has been found to be reduced and significantly altered. Again, this was associated with a more severe disease phenotype. Compromise of the STAT3/Th17 pathway in hyper-IgE syndrome may lead to dysbiosis of the oral microbiota in these patients, causing Candida albicans to switch from commensal to pathogenic. Modification of the microbiota can be used as a therapeutic approach in patients with IEI. Prebiotics, probiotics, postbiotics and fecal microbiota transplantation can be used to restore the balance of the gut microbiota and reduce pathogenicity in IEI patients. Clinical trials are currently underway to understand the impact of this dysbiosis on the phenotype of IEI diseases and its role in their treatment.

Keywords: Immunodeficiency, Microbiome, Microbiota, Fecal microbiota transplantation, Immunoglobulin

Core Tip: Inborn errors of immunity (IEI) disease is associated with microbial dysbiosis and systemic inflammation, especially in the presence of immune dysregulation. Fecal microbiota transplantation in particular, besides not being used as a stand-alone treatment strategy, could be a potential therapeutic tool for dysbiosis in IEI, considering the complications and the complexity of IEI.