Common variable immune deficiency and autoimmunity
Common variable immune deficiency and autoimmunity
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DOI：
10.1016/j.autrev.2006.03.010
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发表时间：
2006-08-01
影响因子：
13.6
通讯作者：
Gershwin, M. Eric
中科院分区：
文献类型：
--
作者：
Brandt, Daniel;Gershwin, M. Eric
常见变异性免疫缺陷（CVID）是一种异质性综合征，以不同程度的低γ球蛋白血症为特征。与许多免疫缺陷疾病类似，自身免疫通常与自身免疫性细胞减少症、结节病样疾病和炎症性肠病有关。最近的研究表明，CVID的选择性免疫缺陷和遗传关联，并表明耐受性丧失的趋势日益增加。这类患者自身免疫性疾病的主要治疗方法通常是高剂量IVIG和皮质类固醇，尽管其他治疗方法，包括tnf - α拮抗剂，也有报道。虽然CVID自身免疫增加的病因尚不清楚，但某些遗传易感性与重复抗原暴露和CVID固有的整体免疫失调相结合可能起着重要作用。(c) 2006 Elsevier B.V.版权所有。
Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by various degrees of hypogammaglobulinemia. Similar to many immunodeficiency disorders, autoimmunity is common with an association with autoimmune cytopenias, a sarcoidosis-like disorder and inflammatory bowel disease. Recent efforts have characterized selective immunological defects and genetic associations in CVID and demonstrate an increased tendency towards loss of tolerance. The mainstay of treatment of automimune disease in such patients is often high dose IVIG and corticosteroids, although other therapies, including TNF-alpha antagonists, have been reported. While the etiology of increased autoimmunity in CVID remains elusive, certain genetic predispositions in combination with repeated antigen exposure and overall immune dysregulation inherent in CVID likely play a significant role. (c) 2006 Elsevier B.V. All fights reserved.