Department of Biosciences and Nutrition, Karolinska Institute, Stockholm, Sweden.
St Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, New York, USA.
J Intern Med. 2023 Aug;294(2):127-144. doi: 10.1111/joim.13628. Epub 2023 Mar 26.
There is growing evidence to suggest that severe disease in children infected with common viruses that are typically benign in other children can result from inborn errors of immunity or their phenocopies. Infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a cytolytic respiratory RNA virus, can lead to acute hypoxemic COVID-19 pneumonia in children with inborn errors of type I interferon (IFN) immunity or autoantibodies against IFNs. These patients do not appear to be prone to severe disease during infection with Epstein-Barr virus (EBV), a leukocyte-tropic DNA virus that can establish latency. By contrast, various forms of severe EBV disease, ranging from acute hemophagocytosis to chronic or long-term illnesses, such as agammaglobulinemia and lymphoma, can manifest in children with inborn errors disrupting specific molecular bridges involved in the control of EBV-infected B cells by cytotoxic T cells. The patients with these disorders do not seem to be prone to severe COVID-19 pneumonia. These experiments of nature reveal surprising levels of redundancy of two different arms of immunity, with type I IFN being essential for host defense against SARS-CoV-2 in respiratory epithelial cells, and certain surface molecules on cytotoxic T cells essential for host defense against EBV in B lymphocytes.
越来越多的证据表明,感染通常对其他儿童无害的常见病毒的儿童如果患有先天性免疫缺陷或免疫缺陷的表型,可能会导致严重疾病。严重急性呼吸综合征冠状病毒 2(SARS-CoV-2)是一种细胞毒性呼吸道 RNA 病毒,可导致 I 型干扰素(IFN)免疫或针对 IFN 的自身抗体先天性缺陷的儿童发生急性低氧血症 COVID-19 肺炎。这些患者在感染嗜淋巴细胞 DNA 病毒 EBV(可建立潜伏感染)时似乎不容易发生严重疾病。相比之下,各种形式的严重 EBV 疾病,从急性噬血细胞性到慢性或长期疾病,如丙种球蛋白血症和淋巴瘤,都可能发生在先天性缺陷的儿童中,这些缺陷会破坏细胞毒性 T 细胞控制 EBV 感染 B 细胞的特定分子桥。这些疾病的患者似乎不容易发生严重的 COVID-19 肺炎。这些自然实验揭示了两种不同免疫途径令人惊讶的冗余水平,I 型 IFN 对呼吸道上皮细胞中 SARS-CoV-2 的宿主防御至关重要,细胞毒性 T 细胞表面的某些分子对 B 淋巴细胞中 EBV 的宿主防御至关重要。