ABSTRACT

Objectives: Common variable immunodeficiency (CVID) is the most common symptomatic inborn error of immunity characterized by variable clinical manifestations.

Methods: Web of Science, Scopus, and PubMed databases were searched systemically to find eligible studies from the earliest available date to February 2020 with standard keywords. Pooled estimates of the autoimmunity prevalence and the corresponding 95% confidence intervals (CI) were calculated using random-effects models.

Results: The overall prevalence of autoimmunity was 29.8% (95% CI: 26.4–33.3; I2 = 82.8%). The prevalences of hematologic autoimmune diseases, autoimmune gastrointestinal disorders, autoimmune rheumatologic disorders, autoimmune skin disorders, and autoimmune endocrinopathy in CVID patients were 18.9%, 11.5%, 6.4%, 5.9%), and 2.5%, respectively. There were significantly higher lymphocyte, CD3 + T cell, and CD4 + T cell count among CVID patients without autoimmunity (p< 0.05). Furthermore, failure to thrive, organomegaly, enteropathy, and meningitis was significantly higher in CVID patients with autoimmunity(p< 0.05).

Conclusions: Many CVID patients could present with autoimmunity as part of the disease or even as the first or only clinical manifestation of the disease. Care providers may need to pay particular attention to the possible association of these two disorders since the co-occurrence of CVID and autoimmunity could be a misleading clue.

摘要

目的：常见变异免疫缺陷（CVID）是最常见的有症状的先天免疫缺陷，其特点是临床表现多变。

方法：系统搜索 Web of Science、Scopus 和 PubMed 数据库，以使用标准关键字查找从最早可用日期到 2020 年 2 月的符合条件的研究。使用随机效应模型计算自身免疫流行率和相应的 95% 置信区间 (CI) 的汇总估计值。

结果：自身免疫的总体患病率为 29.8%（95% CI：26.4-33.3；I2 = 82.8%）。CVID患者血液自身免疫性疾病、自身免疫性胃肠病、自身免疫性风湿病、自身免疫性皮肤病和自身免疫性内分泌病的患病率分别为18.9%、11.5%、6.4%、5.9%和2.5%。无自身免疫的 CVID 患者淋巴细胞、CD3 + T 细胞和 CD4 + T 细胞计数显着升高（p < 0.05）。此外，自身免疫性CVID患者发育不良、器官肿大、肠病和脑膜炎的发生率显着升高（p < 0.05）。

结论：许多 CVID 患者可能表现为自身免疫作为疾病的一部分，甚至作为疾病的第一个或唯一的临床表现。护理提供者可能需要特别注意这两种疾病的可能关联，因为 CVID 和自身免疫的同时发生可能是一个误导性的线索。