Brandt Daniel, Gershwin M Eric
Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 E. Health Sciences Drive, Suite 6510, Davis, CA 95616, USA.
Autoimmun Rev. 2006 Aug;5(7):465-70. doi: 10.1016/j.autrev.2006.03.010. Epub 2006 Apr 24.
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 autoimmune 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.
普通可变免疫缺陷（CVID）是一种异质性综合征，其特征为不同程度的低丙种球蛋白血症。与许多免疫缺陷疾病相似，自身免疫很常见，与自身免疫性血细胞减少、类肉瘤样疾病和炎症性肠病有关。最近的研究已明确了CVID中的选择性免疫缺陷和基因关联，并表明其耐受性丧失的倾向增加。这类患者自身免疫性疾病的主要治疗方法通常是大剂量静脉注射免疫球蛋白（IVIG）和皮质类固醇，不过也有报道称包括肿瘤坏死因子-α拮抗剂在内的其他疗法。虽然CVID中自身免疫增加的病因仍不清楚，但某些遗传易感性与反复接触抗原以及CVID固有的整体免疫失调可能起了重要作用。
