期刊
WORLD ALLERGY ORGANIZATION JOURNAL
卷 14, 期 2, 页码 -出版社
ELSEVIER
DOI: 10.1016/j.waojou.2021.100513
关键词
Inborn errors of immunity; Primary immunodeficiency; Atopy; Atopic phenotypes; Allergy
资金
- Italian Society of Pediatric Allergy and Immunology (SIAIP)
Inborn errors of immunity (IEI) are a group of disorders caused by mutations in genes associated with immune regulation and defense, leading to various symptoms including recurrent infections, autoimmunity, inflammatory manifestations, lymphoproliferation, and malignancy. It is increasingly recognized that common allergic symptoms could also indicate underlying immunodeficiency and/or immune dysregulation.
Inborn errors of immunity (IEI) are a heterogeneous group of disorders, mainly resulting from mutations in genes associated with immunoregulation and immune host defense. These disorders are characterized by different combinations of recurrent infections, autoimmunity, inflammatory manifestations, lymphoproliferation, and malignancy. Interestingly, it has been increasingly observed that common allergic symptoms also can represent the expression of an underlying immunodeficiency and/or immune dysregulation. Very high IgE levels, peripheral or organ-specific hypereosinophilia, usually combined with a variety of atopic symptoms, may sometimes be the epiphenomenon of a monogenic disease. Therefore, allergists should be aware that severe and/or therapy-resistant atopic disorders might be the main clinical phenotype of some IEI. This could pave the way to target therapies, leading to better quality of life and improved survival in affected patients.
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