Immune dysregulation.
J Allergy Clin Immunol
; 151(1): 70-80, 2023 01.
Article
em En
| MEDLINE
| ID: mdl-36608984
ABSTRACT
The understanding of immune dysregulation in many different diseases continues to grow. There is increasing evidence that altered microbiome and gut barrier dysfunction contribute to systemic inflammation in patients with primary immunodeficiency and in patients with rheumatic disease. Recent research provides insight into the process of induction and maturation of pathogenic age-associated B cells and highlights the role of age-associated B cells in creating tissue inflammation. T follicular regulatory cells are shown to help maintain B-cell tolerance, and therapeutic approaches to increase or promote T follicular regulatory cells may help prevent or decrease immune dysregulation. Meanwhile, novel studies of systemic-onset juvenile idiopathic arthritis reveal a strong HLA association with interstitial lung disease and identify key aspects of the pathogenesis of macrophage activation syndrome. Studies of hyperinflammatory syndromes, including the recently described multisystem inflammatory syndrome of children, characterize similarities and differences in cytokine profiles and T-cell activation. This review focuses on recent advances in the understanding of immune dysregulation and describes potential key factors that may function as biomarkers for disease or targets for therapeutic interventions. Future trials are necessary to address the many remaining questions with regards to pathogenesis, diagnosis, and treatment of autoimmune, inflammatory, and immunodeficiency syndromes.
Palavras-chave
Barrier dysfunction; DAD2; Epstein-Barr virus; Kawasaki syndrome; Schnitzler; VEXAS; common variable immune deficiency; drug reaction with eosinophilia and systemic symptoms; dysbiosis; hemophagocytic lymphocytic histiocytosis; macrophage activation syndrome; multisystem inflammatory syndrome in children; rheumatoid arthritis; systemic-onset juvenile idiopathic arthritis
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Artrite Juvenil
/
Doenças Reumáticas
/
Síndrome de Ativação Macrofágica
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Síndromes de Imunodeficiência
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Child
/
Humans
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article