Shamriz Oded, Tal Yuval, Talmon Aviv, Nahum Amit
Allergy and Clinical Immunology Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
The Lautenberg Center for Immunology and Cancer Research, Institute of Medical Research Israel-Canada, Hebrew University-Hadassah Medical School, Jerusalem, Israel.
Front Immunol. 2020 Oct 16;11:593289. doi: 10.3389/fimmu.2020.593289. eCollection 2020.
Children with chronic mucocutaneous candidiasis (CMC) experience recurrent infections with . Moreover, immune dysregulation in the early life of these patients induces various autoimmune diseases and affects normal growth and development. The adaptive and innate immune system components play a significant role in anti-fungal response. This response is mediated through IL-17 production by T helper cells. Inborn errors in IL-17-mediated pathways or . sensing molecules are known to cause CMC. In this review, we describe underlying immune mechanisms of monogenic primary immune deficiency disorders known to cause CMC. We will explore insights into current management of these patients and novel available therapies.
患有慢性黏膜皮肤念珠菌病(CMC)的儿童会反复感染念珠菌。此外,这些患者早年的免疫失调会引发各种自身免疫性疾病,并影响正常的生长发育。适应性和先天性免疫系统成分在抗真菌反应中发挥着重要作用。这种反应是通过辅助性T细胞产生白细胞介素-17来介导的。已知白细胞介素-17介导途径或传感分子中的先天性错误会导致CMC。在这篇综述中,我们描述了已知会导致CMC的单基因原发性免疫缺陷疾病的潜在免疫机制。我们将探讨这些患者当前管理方法的见解以及新的可用疗法。