Clinical Immunology Service, National Institute of Pediatrics, Mexico City, Mexico.
Allergy and Immunology Department, Hospital de Especialidades, National Medical Center, Siglo XXI" IMSS, Mexico City, Mexico.
J Clin Immunol. 2021 Oct;41(7):1463-1478. doi: 10.1007/s10875-021-01077-5. Epub 2021 Jun 10.
Patients with inborn errors of immunity (IEI) have a compromised or inappropriate immune response. Although they might be considered a high-risk group for severe SARS-CoV-2 infection, the reported impact of COVID-19 in these patients has been reassuring, while the differential susceptibility of distinct types of IEI remains unclear.
We aimed to describe the findings and outcomes of our known patients with IEI who were diagnosed with COVID-19.
In a retrospective study from March 2020 to February 2021, four centers in Mexico collected clinical, laboratory, and genetic data from pediatric and adult patients with known diagnoses of IEI who presented with COVID-19, based on compatible symptoms and positive SARS-CoV-2 testing or known household exposure.
We report 31 patients with known IEI from Mexico who presented with SARS-CoV-2 infection. Seventy-four percent were male, 52% were pediatric, and 81% survived. Their ages ranged from 5 months to 56 years, with a median of 17 years. Sixty-five percent had predominant antibody deficiencies, 48% were hospitalized, and 26% required ICU. Pediatric patients had a higher hospital admission rate than adults. Inpatient mortality was 40%, and ICU mortality rate was 63%. Forty-eight percent developed pneumonia, while 36% had evidence of hyperinflammation (4 adults and 7 children). Predominant laboratory features were lymphopenia and thrombocytopenia, seen in 70 and 44% of patients, respectively. The serum D-dimer median value was 2.6 (0.5-20.6) μg/mL, and the median highest ferritin value was 1015 (32-10,303) ng/mL. Intravenous immunoglobulin was used in 80% of patients. Other treatments included macrolides (39%) and corticosteroids (29%). Six patients died from secondary infection or uncontrolled systemic inflammation.
Although impaired immunity due to IEI may be a predisposing factor for severe COVID-19, most of our patients with IEI who acquired the SARS-CoV-2 infection developed a well-tolerated infection and survived, as have more than 80% of worldwide reported patients to date. An impaired immune or inflammatory response may be a predisposing factor for some and a protective factor for others. A systematic review of the literature could help identify those patients at risk of severe disease and complications. Healthcare-associated infections should be aggressively prevented.
患有先天性免疫缺陷（IEI）的患者免疫反应受损或不适当。尽管他们可能被认为是感染严重 SARS-CoV-2 的高风险人群，但迄今为止，COVID-19 在这些患者中的影响是令人安心的，而不同类型的 IEI 的易感性差异尚不清楚。
我们旨在描述在墨西哥的四个中心确诊患有 IEI 的患者的 COVID-19 发现和结局。
在 2020 年 3 月至 2021 年 2 月期间，我们进行了一项回顾性研究，从墨西哥的四个中心收集了已知患有 IEI 的儿科和成年患者的临床、实验室和遗传数据，这些患者出现了 COVID-19 症状，检测结果呈 SARS-CoV-2 阳性或有已知的家庭接触史。
我们报告了 31 名来自墨西哥的已知 IEI 患者感染了 SARS-CoV-2。74%为男性，52%为儿科患者，81%存活。年龄从 5 个月到 56 岁，中位数为 17 岁。65%的患者存在主要的抗体缺陷，48%住院，26%需要 ICU 治疗。儿科患者的住院率高于成人。住院死亡率为 40%，ICU 死亡率为 63%。48%的患者患有肺炎，而 36%的患者存在高炎症反应（4 名成人和 7 名儿童）。主要的实验室特征是淋巴细胞减少和血小板减少，分别有 70%和 44%的患者出现。血清 D-二聚体中位数为 2.6（0.5-20.6）μg/ml，最高铁蛋白中位数为 1015（32-10303）ng/ml。80%的患者使用了静脉注射免疫球蛋白。其他治疗包括大环内酯类（39%）和皮质类固醇（29%）。6 名患者因继发感染或失控的全身炎症而死亡。
尽管 IEI 导致的免疫受损可能是感染严重 COVID-19 的一个诱发因素，但我们大多数感染 SARS-CoV-2 的 IEI 患者的感染都得到了良好的耐受，并且存活下来了，这与迄今为止全球报告的 80%以上的患者一样。免疫或炎症反应受损可能是某些患者的诱发因素，也是另一些患者的保护因素。对文献进行系统回顾可能有助于识别那些有患严重疾病和并发症风险的患者。应积极预防与医疗保健相关的感染。
