Common variable immunodeficiency (CVID), although the most common primary immunodeficiency in humans, is a rare disease. We explored the spatial global distribution and country-wise prevalence of CVID, based on published data and those available from databases. As a country’s medical progress is linked to its technological and socio-economic developmental status, we expected that observed CVID prevalence was linked to human wellbeing. To assess this, we examined the correlation of observed CVID prevalence and the UNDP Human Development Index (HDI), which is a key measure of human development. Seventy-four data sets from 47 countries were available (most of them no older than 10 years). Analyses revealed that observed CVID prevalence ranged from 0.001 to 3.374 per 100,000 (mean ) and was highest in “high” HDI countries (). Observed prevalence was particularly high in countries where immunodeficiencies are systematically documented in registers. In “low” and “middle” HDI countries, CVID awareness is extremely poor. Assuming that true CVID prevalence does not differ among countries, this study, though preliminary, provides evidence that the discrepancy between observed and (unknown) true prevalence can be clearly linked to the countries’ developmental status. As a potential alternative explanation, we briefly discuss the possibility that variation in CVID prevalence is related to human genetic lineage.

中文翻译：

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根据联合国开发计划署人类发展指数（HDI），全球通用可变免疫缺陷（CVID）的全球分布：一种罕见疾病的初步观点。

尽管人类中最常见的原发性免疫缺陷，但常见的可变免疫缺陷症（CVID）是一种罕见的疾病。我们基于已发布的数据和数据库中的可用数据，探索了CVID的空间全球分布和国家/地区流行率。由于一个国家的医学进步与其技术和社会经济发展状况息息相关，因此我们希望观察到的CVID患病率与人类福祉息息相关。为了评估这一点，我们检查了观察到的CVID患病率与开发计划署人类发展指数（HDI）的相关性，后者是人类发展的关键指标。有来自47个国家的74个数据集（大多数不超过10年）。分析显示，观察到的CVID患病率范围为每10万0.001到3.374（平均），在“高” HDI国家（）。在登记册中系统记录免疫缺陷的国家中，观察到的患病率特别高。在“低”和“中”的人类发展指数国家中，对CVID的认识极差。假设真正的CVID患病率在各个国家之间没有差异，该研究尽管是初步的，但提供的证据表明，观察到的和（未知）真实患病率之间的差异可以明确地与各国的发展状况联系起来。作为一种可能的替代解释，我们简要讨论CVID患病率变异与人类遗传谱系有关的可能性。