Immune tolerance breakdown in inborn errors of immunity: Paving the way to novel therapeutic approaches

Up to 25% of the patients with inborn errors of immunity (IEI) also exhibit immunodysregulatory features. The association of immune dysregulation and immunodeficiency may be explained by different mechanisms. The understanding of mechanisms underlying immune dysregulation in IEI has paved the way fo...

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Published in:Clinical immunology (Orlando, Fla.) Fla.), 2023-06, Vol.251, p.109302-109302, Article 109302
Main Authors: Giardino, Giuliana, Romano, Roberta, Lougaris, Vassilios, Castagnoli, Riccardo, Cillo, Francesca, Leonardi, Lucia, La Torre, Francesco, Soresina, Annarosa, Federici, Silvia, Cancrini, Caterina, Pacillo, Lucia, Toriello, Elisabetta, Cinicola, Bianca Laura, Corrente, Stefania, Volpi, Stefano, Marseglia, Gian Luigi, Pignata, Claudio, Cardinale, Fabio
Format: Article
Language:English
Subjects:
Autoimmunity
Humans
Immune System Diseases - genetics
Immune System Diseases - therapy
Immune Tolerance
Immunodysregulation
Inborn errors of immunity
T Reg
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Summary:Up to 25% of the patients with inborn errors of immunity (IEI) also exhibit immunodysregulatory features. The association of immune dysregulation and immunodeficiency may be explained by different mechanisms. The understanding of mechanisms underlying immune dysregulation in IEI has paved the way for the development of targeted treatments. In this review article, we will summarize the mechanisms of immune tolerance breakdown and the targeted therapeutic approaches to immune dysregulation in IEI. •Immunodysregulatory features may be observed in about 25% of patients with Inborn errors of immunity.•The association of immune dysregulation and immunodeficiency may be explained by different mechanisms.•The identification of the pathogenetic mechanisms has led to the development of precision treatments.
ISSN:1521-6616
1521-7035
DOI:10.1016/j.clim.2023.109302