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


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.

SUBMITTER: Krzyzanowski D 

PROVIDER: S-EPMC10283154 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature

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

Krzyzanowski Damian D   Oszer Aleksandra A   Madzio Joanna J   Zdunek Maciej M   Kolodrubiec Julia J   Urbanski Bartosz B   Mlynarski Wojciech W   Janczar Szymon S  

Frontiers in immunology 20230607


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  ...[more]

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