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Review
. 2023 Jun 7:14:1128581.
doi: 10.3389/fimmu.2023.1128581. eCollection 2023.

The paradox of autoimmunity and autoinflammation in inherited neutrophil disorders - in search of common patterns

Damian Krzyzanowski  1   2 , Aleksandra Oszer  1 , Joanna Madzio  1 , Maciej Zdunek  1 , Julia Kolodrubiec  1 , Bartosz Urbanski  1 , Wojciech Mlynarski  1 , Szymon Janczar  1
Affiliations
Review

The paradox of autoimmunity and autoinflammation in inherited neutrophil disorders - in search of common patterns

Damian Krzyzanowski et al. Front Immunol. .

Abstract

Congenital defects of neutrophil number or function are associated with a severe infectious phenotype that may require intensive medical attention and interventions to be controlled. While the infectious complications in inherited neutrophil disorders are easily understood much less clear and explained are autoimmune and autoinflammatory phenomena. We survey the clinical burden of autoimmunity/autoinflammation in this setting, search for common patterns, discuss potential mechanisms and emerging treatments.

Keywords: autoimmunity; autoinflammation; chronic granulomatous disease; inflammatory bowel disease; neutropenia; severe congenital neutropenia.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Proposed common mechanisms for increased autoimmunity and autoinflammation in inherited neutrophil disorders. CGD, chronic granulomatous disease; DAMPs, damage-associated molecular patterns; PS, phosphatidylserine; PSR, specific phosphatidylserine receptor; APC, antigen presenting cell; MHC, major histocompatibility complex; TCR, T-cell receptor; NADPH, nicotinamide adenine dinucleotide phosphate; GSDMD, gasdermin D; FOXP3, forkhead box P3; 1,5AG6P, 1,5-anhydroglucitol-6-phosphate; G6P, glucose 6-phosphate; G6PC3, glucose-6-phosphatase, catalytic 3; Pi, inorganic phosphate; GLUT2, glucose transporter 2; NPT4, solute carrier family 17 (organic anion transporter), member 3; ER, endoplasmic reticulum.
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