Saba Fekrvand, Kiarash Saleki, Hassan Abolhassani, Amir Almasi-Hashiani, Ali Hakimelahi, Nikan Zargarzadeh, Mir Saeed Yekaninejad, Nima Rezaei
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The majority of patients had critical infections (1013 cases, 27.8%). The highest frequency of critical and severe cases was observed in phenocopies of IEI (95.2%), defects in intrinsic and innate immunity (69.4%) and immune dysregulation (23.9%). 446 cases (12.2%) succumbed to the disease and the highest mortality was observed in IEI phenocopies (34.6%). COVID-19 frequency in immunodeficient patients was 11.9% (95% CI: 8.3 to 15.5%) with innate immunodeficiency having the highest COVID-19 frequency [34.1% (12.1 to 56.0%)]. COVID-19 case fatality rate among IEI patients was estimated as 5.4% (95% CI: 3.5-8.3%, <i>n</i> = 8 studies, I2 = 17.5%).</p><p><strong>Conclusion: </strong>IEI with underlying defects in specific branches of the immune system responding to RNA virus infection experience a higher frequency and mortality of COVID-19 infection. Increasing awareness about these entities and underlying genetic defects, adherence to prophylactic strategies and allocating more clinical attention to these patients could lead to a decrease in COVID-19 frequency and mortality in these patients.</p>","PeriodicalId":73372,"journal":{"name":"Infectious diseases (London, England)","volume":" ","pages":"1-35"},"PeriodicalIF":0.0000,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"COVID-19 infection in inborn errors of immunity and their phenocopies: a systematic review and meta-analysis.\",\"authors\":\"Saba Fekrvand, Kiarash Saleki, Hassan Abolhassani, Amir Almasi-Hashiani, Ali Hakimelahi, Nikan Zargarzadeh, Mir Saeed Yekaninejad, Nima Rezaei\",\"doi\":\"10.1080/23744235.2025.2483339\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Inborn errors of immunity (IEI) are congenital disorders of the immune system. 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引用次数: 0
摘要
背景:先天性免疫错误(IEI)是免疫系统的先天性疾病。由于免疫系统受损,与普通人群相比,他们出现更严重的COVID-19病程的风险更高。目的:在此,我们旨在系统地回顾感染SARS-CoV-2的IEI患者的各个方面。此外,我们进行了一项荟萃分析,以确定不同IEI患者中COVID-19的频率。方法:检索Embase、Web of Science、PubMed和Scopus,引入IEI和COVID-19相关术语。结果:共纳入3646例具有COVID-19感染史的IEI病例。危重感染占多数(1013例,27.8%)。在IEI表型(95.2%)、固有免疫和先天免疫缺陷(69.4%)和免疫失调(23.9%)中,危重病例的发生率最高。446例(12.2%)死亡,IEI表型死亡率最高(34.6%)。免疫缺陷患者的COVID-19频率为11.9% (95% CI: 8.3 ~ 15.5%),先天免疫缺陷患者的COVID-19频率最高[34.1%(12.1 ~ 56.0%)]。IEI患者的COVID-19病死率估计为5.4% (95% CI: 3.5-8.3%, n = 8项研究,I2 = 17.5%)。结论:在应对RNA病毒感染的免疫系统特定分支存在潜在缺陷的IEI患者发生COVID-19感染的频率和死亡率更高。提高对这些实体和潜在遗传缺陷的认识,坚持预防策略,并为这些患者分配更多的临床关注,可能会降低这些患者的COVID-19发病率和死亡率。
COVID-19 infection in inborn errors of immunity and their phenocopies: a systematic review and meta-analysis.
Background: Inborn errors of immunity (IEI) are congenital disorders of the immune system. Due to impaired immune system, they are at a higher risk to develop a more severe COVID-19 course compared to general population.
Objectives: Herein, we aimed to systematically review various aspects of IEI patients infected with SARS-CoV-2. Moreover, we performed a meta-analysis to determine the frequency of COVID-19 in patients with different IEI.
Methods: Embase, Web of Science, PubMed, and Scopus were searched introducing terms related to IEI and COVID-19.
Results: 3646 IEI cases with a history of COVID-19 infection were enrolled. The majority of patients had critical infections (1013 cases, 27.8%). The highest frequency of critical and severe cases was observed in phenocopies of IEI (95.2%), defects in intrinsic and innate immunity (69.4%) and immune dysregulation (23.9%). 446 cases (12.2%) succumbed to the disease and the highest mortality was observed in IEI phenocopies (34.6%). COVID-19 frequency in immunodeficient patients was 11.9% (95% CI: 8.3 to 15.5%) with innate immunodeficiency having the highest COVID-19 frequency [34.1% (12.1 to 56.0%)]. COVID-19 case fatality rate among IEI patients was estimated as 5.4% (95% CI: 3.5-8.3%, n = 8 studies, I2 = 17.5%).
Conclusion: IEI with underlying defects in specific branches of the immune system responding to RNA virus infection experience a higher frequency and mortality of COVID-19 infection. Increasing awareness about these entities and underlying genetic defects, adherence to prophylactic strategies and allocating more clinical attention to these patients could lead to a decrease in COVID-19 frequency and mortality in these patients.