Introduction

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.

Objective

We aimed to describe the findings and outcomes of our known patients with IEI who were diagnosed with COVID-19.

Methods

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.

Results

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.

Discussion

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.

中文翻译：

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先天免疫错误背景下的 COVID-19：来自墨西哥的 31 名患者的病例系列

介绍

患有先天性免疫缺陷 (IEI) 的患者具有受损或不适当的免疫反应。尽管他们可能被认为是严重 SARS-CoV-2 感染的高危人群，但据报道 COVID-19 对这些患者的影响令人放心，而不同类型 IEI 的不同易感性仍不清楚。

客观的

我们旨在描述我们已知的被诊断为 COVID-19 的 IEI 患者的发现和结果。

方法

在 2020 年 3 月至 2021 年 2 月的一项回顾性研究中，墨西哥的四个中心根据兼容的症状和阳性 SARS-CoV-收集了已知诊断为 IEI 并出现 COVID-19 的儿科和成人患者的临床、实验室和基因数据。 2 测试或已知的家庭接触。

结果

我们报告了来自墨西哥的 31 名已知 IEI 患者，他们出现了 SARS-CoV-2 感染。74% 为男性，52% 为儿童，81% 存活。他们的年龄从5个月到56岁不等，中位数为17岁。65% 有主要抗体缺陷，48% 住院，26% 需要 ICU。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% 的人患上了肺炎，而 36% 的人有过度炎症的证据（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，分别见于 70% 和 44% 的患者。血清 D-二聚体中值为 2.6 (0.5–20.6) μg/mL，中位最高铁蛋白值为 1015 (32–10,303) ng/mL。80% 的患者使用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%）。六名患者死于继发感染或不受控制的全身炎症。

讨论

尽管 IEI 导致的免疫力受损可能是严重 COVID-19 的诱发因素，但我们大多数获得 SARS-CoV-2 感染的 IEI 患者都出现了良好的感染耐受性并存活了下来，据报道，全球超过 80%患者至今。免疫或炎症反应受损可能是某些人的诱发因素和其他人的保护因素。对文献进行系统回顾可以帮助识别那些有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。