Clinical Immunology Service, Institute of Immunology and Immunotherapy, University of Birmingham, UK.
Lancashire Teaching Hospitals NHS Foundation Trust, Preston, Lancashire, UK.
Clin Exp Immunol. 2022 Sep 29;209(3):247-258. doi: 10.1093/cei/uxac008.
In March 2020, the United Kingdom Primary Immunodeficiency Network (UKPIN) established a registry of cases to collate the outcomes of individuals with PID and SID following SARS-CoV-2 infection and treatment. A total of 310 cases of SARS-CoV-2 infection in individuals with PID or SID have now been reported in the UK. The overall mortality within the cohort was 17.7% (n = 55/310). Individuals with CVID demonstrated an infection fatality rate (IFR) of 18.3% (n = 17/93), individuals with PID receiving IgRT had an IFR of 16.3% (n = 26/159) and individuals with SID, an IFR of 27.2% (n = 25/92). Individuals with PID and SID had higher inpatient mortality and died at a younger age than the general population. Increasing age, low pre-SARS-CoV-2 infection lymphocyte count and the presence of common co-morbidities increased the risk of mortality in PID. Access to specific COVID-19 treatments in this cohort was limited: only 22.9% (n = 33/144) of patients admitted to the hospital received dexamethasone, remdesivir, an anti-SARS-CoV-2 antibody-based therapeutic (e.g. REGN-COV2 or convalescent plasma) or tocilizumab as a monotherapy or in combination. Dexamethasone, remdesivir, and anti-SARS-CoV-2 antibody-based therapeutics appeared efficacious in PID and SID. Compared to the general population, individuals with PID or SID are at high risk of mortality following SARS-CoV-2 infection. Increasing age, low baseline lymphocyte count, and the presence of co-morbidities are additional risk factors for poor outcome in this cohort.
2020 年 3 月,英国原发性免疫缺陷网络(UKPIN)建立了一个病例登记处,以整理在 SARS-CoV-2 感染和治疗后患有 PID 和 SID 的个体的结果。目前,英国已报告了 310 例 PID 或 SID 个体的 SARS-CoV-2 感染病例。该队列的总死亡率为 17.7%(n=55/310)。CVID 患者的感染病死率(IFR)为 18.3%(n=17/93),接受 IgRT 的 PID 患者的 IFR 为 16.3%(n=26/159),SID 患者的 IFR 为 27.2%(n=25/92)。PID 和 SID 患者的住院死亡率更高,死亡年龄也比一般人群年轻。年龄增长、SARS-CoV-2 感染前淋巴细胞计数低以及存在常见合并症增加了 PID 的死亡风险。本队列中 COVID-19 治疗的可及性有限:仅 22.9%(n=33/144)住院患者接受了地塞米松、瑞德西韦、抗 SARS-CoV-2 抗体治疗(如 REGN-COV2 或恢复期血浆)或托珠单抗单药或联合治疗。地塞米松、瑞德西韦和抗 SARS-CoV-2 抗体治疗在 PID 和 SID 中似乎有效。与一般人群相比,PID 或 SID 患者在 SARS-CoV-2 感染后死亡风险较高。年龄增长、基线淋巴细胞计数低和合并症的存在是该队列预后不良的其他危险因素。