Abstract：
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an inborn error of immunity affecting both multiple endocrine organs and susceptibility to candidiasis, each with an autoimmune basis. Recently, high titer neutralizing anti-type I interferon (IFN) autoantibodies have been linked with increased severity of SARS-CoV-2 and varicella zoster virus infections in APECED patients. Examining immunity against cytomegalovirus (CMV), we found a higher prevalence of anti-CMV IgG antibodies in patients with APECED (N = 19) than in 44 healthy controls (90% vs 64%, p = 0.04); the similar difference in their IgG levels did not achieve significance (95 ± 74 vs 64 ± 35 IU/mL, ns.). In contrast, the frequency of CMV-specific T cells was lower (804 ± 718/million vs 1591 ± 972/million PBMC p = 0.03). We saw no correlations between levels of anti-CMV IgG and anti-IFN antibodies in APECED patients or in a separate cohort of patients with thymoma (n = 70), over 60% of whom also had anti-IFN antibodies. Our results suggest a dysregulated response to CMV in APECED patients and highlight immunodeficiency to viral infections as part of the disease spectrum.
摘要：
自身免疫性多内分泌病-念珠菌病-外胚层营养不良（APECED）是一种先天性免疫错误，影响多种内分泌器官和念珠菌病的易感性。每个都有自身免疫基础。最近,高滴度中和性抗I型干扰素(IFN)自身抗体与APECED患者中SARS-CoV-2和水痘带状疱疹病毒感染的严重程度增加有关.检查对巨细胞病毒（CMV）的免疫力，我们发现APECED患者（N=19）的抗CMVIgG抗体患病率高于44名健康对照（90％vs64％，p=0.04）；它们的IgG水平相似的差异没有达到显著性（95±74vs64±35IU/mL，.).相比之下,CMV特异性T细胞的频率较低（804±718/百万对1591±972/百万PBMCp=0.03）。我们在APECED患者或单独的胸腺瘤患者队列中发现抗CMVIgG和抗IFN抗体水平之间没有相关性（n=70），超过60％的人也有抗IFN抗体。我们的结果表明，APECED患者对CMV的反应失调，并强调了病毒感染的免疫缺陷是疾病谱的一部分。
