Autoimmunity in common variable immunodeficiency
Autoimmunity in common variable immunodeficiency
复制标题
DOI：
10.1007/s10875-008-9172-9
复制
发表时间：
2008-05-01
影响因子：
9.1
通讯作者：
Rizzo, Luiz Vicente
中科院分区：
文献类型：
--
作者：
Lopes-da-Silva, Susana;Rizzo, Luiz Vicente
CVID是成年期最常见的有症状的原发性免疫缺陷。不同的作者报道CVID中自身免疫性疾病的高患病率，并且已经提出了几种机制来解释这种明显的矛盾。遗传易感性，根据目前的监测，先天性和适应性免疫缺陷导致持续/复发性感染，免疫失调的程度不同，以及可能的失败，在中央和外周机制的耐受性诱导或维护可能都有助于增加autoimmunity.Conclusions数据的临床/免疫学资料受影响的患者和治疗大多是关于自身免疫性血细胞减少症，CVID中最常见的自身免疫性疾病。治疗基于传统的替代方案，并结合使用新药物（包括利妥昔单抗和英夫利西单抗）的短暂经验。早期免疫球蛋白替代治疗的益处和假设的自身免疫性过早预测因子作为CVID患者随访的潜在改善进行了讨论。
immunodeficiency (CVID), the most common symptomatic primary immunodeficiency in adulthood. Different authors report high prevalences of autoimmune diseases in CVID, and several mechanisms have been proposed to explain this apparent paradox. Genetic predisposition, under current surveillance, innate and adaptive immunity deficiencies leading to persistent/recurrent infections, variable degrees of immune dysregulation, and possible failure in central and peripheral mechanisms of tolerance induction or maintenance may all contribute to increased autoimmunity.Conclusions Data on the clinical/immunological profile of affected patients and treatment are available mostly concerning autoimmune cytopenias, the most common autoimmune diseases in CVID. Treatment is based on conventional alternatives, in association with short experience with new agents, including rituximab and infliximab. Benefits of early immunoglobulin substitutive treatment and hypothetical premature predictors of autoimmunity are discussed as potential improvements to CVID patients' follow-up.