Cellular and Molecular Mechanisms Breaking Immune Tolerance in Inborn Errors of Immunity

Overview

Journal Cell Mol Immunol

Date 2021 Apr 2

PMID 33795850

Citations 31

Authors

Georgios Sogkas

Faranaz Atschekzei

Ignatius Ryan Adriawan

Natalia Dubrowinskaja

Torsten Witte

Reinhold Ernst Schmidt

Affiliations

Soon will be listed here.

Abstract

In addition to susceptibility to infections, conventional primary immunodeficiency disorders (PIDs) and inborn errors of immunity (IEI) can cause immune dysregulation, manifesting as lymphoproliferative and/or autoimmune disease. Autoimmunity can be the prominent phenotype of PIDs and commonly includes cytopenias and rheumatological diseases, such as arthritis, systemic lupus erythematosus (SLE), and Sjogren's syndrome (SjS). Recent advances in understanding the genetic basis of systemic autoimmune diseases and PIDs suggest an at least partially shared genetic background and therefore common pathogenic mechanisms. Here, we explore the interconnected pathogenic pathways of autoimmunity and primary immunodeficiency, highlighting the mechanisms breaking the different layers of immune tolerance to self-antigens in selected IEI.

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