Open AccessThe Heterogeneous Pathogenesis of Selective Immunoglobulin A Deficiency
Author(s) -
Yasser Bagheri,
Roozbeh Sanaei,
Reza Yazdani,
Mehdi Shekarabi,
Reza Falak,
Javad Mohammadi,
Hassan Abolhassani,
Asghar Aghamohammadi
Publication year - 2019
Publication title -
international archives of allergy and immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.696
H-Index - 100
eISSN - 1423-0097
pISSN - 1018-2438
DOI - 10.1159/000499044
Subject(s) - immunology , biology , major histocompatibility complex , pathogenesis , antibody , primary immunodeficiency , immunoglobulin a , b cell , immune system , immunoglobulin g
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.