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. 2019 Dec 10;10:2753. doi: 10.3389/fimmu.2019.02753

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Copyright © 2019 Gereige and Maglione.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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B-cell and T-cell dysregulation and immunophenotypic characteristics of autoimmunity in CVID. Some features of B-cell dysregulation in CVID patients with autoimmunity include elevated CD21^low B cells and IgM and increased autoreactive B cells in the periphery. On the other hand, memory B-cells, and B-cell receptor diversity are decreased. Disturbance of T-cell homeostasis, with a decrease in T regulatory cells (T_R) and a skew toward type 1 immunity, has been associated with autoimmunity in CVID. Factors that have been identified in CVID patients which lead to downregulation of T_R cells include LRBA and CTLA-4 deficiency, as well as IgA deficiency. Upregulation of mTOR activity in GOF PIK3CD may also promote this immune dysregulation, while STAT3 GOF leads to expansion of T_H17. BAFF augments T_H1 cytokine production via its effects on the BAFF receptor on T cells, and promotes the survival of autoreactive antibodies via its receptor on B cells.