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The Heterogeneous Pathogenesis of Selective Immunoglobulin a Deficiency Publisher Pubmed



Bagheri Y1, 2, 3 ; Sanaei R4, 5 ; Yazdani R2 ; Shekarabi M3, 4 ; Falak R3, 4 ; Mohammadi J6 ; Abolhassani H2, 7 ; Aghamohammadi A2
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Source: International Archives of Allergy and Immunology Published:2019


Abstract

Selective immunoglobulin A deficiency (SIgAD) is the most prevalent type of primary immunodeficiency disorder. The phenotypic feature of SIgAD is related to a defect in B lymphocyte differentiation into plasma cell-producing immunoglobulin A (IgA). In this review, we summarize the recent advances in this regard. Genetic (including major histocompatibility complex [MHC] and non-MHC genes), immunologic (including B and T lymphocyte subsets abnormality), cytokines/chemokines and their related receptors, apoptosis and microbiota defects are reviewed. The mechanisms leading to SIgAD are most likely multifactorial and it can be speculated that several pathways controlling B cells functions or regulating epigenetic of the IGHA gene encoding constant region of IgA heavy chain and long-term survival of IgA switched memory B cells and plasma cells may be defective in different SIgAD patients. © 2019 S. Karger AG, Basel.
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