Immunoregulation Section, Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), Intramural Research Program, National Institutes of Health (NIH), Bethesda, MD 20892, USA.
HIV Pathogenesis Section, Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases/National Institutes of Health, Bethesda, MD, USA.
Clin Immunol. 2021 Mar;224:108664. doi: 10.1016/j.clim.2021.108664. Epub 2021 Jan 7.
Characterize autoantibodies and autoimmune diseases in a prospective cohort of patients with Idiopathic CD4 Lymphocytopenia (ICL) a rare immunodeficiency characterized by an absolute CD4 T count of <300 cells/μl in the absence of HIV or HTLV infection.
Single-Center prospective study of 67 patients conducted over an 11-year period. Rheumatologic evaluation and measurement of autoantibodies were systematically conducted, and flow cytometry of immune cell subsets was performed in a subset of patients.
54% of referred patients had clinical evidence of autoimmunity, with 34% having at least one autoimmune disease, most commonly autoimmune thyroid disease. 19%, had autoantibodies or incomplete features of autoimmune disease. Patients with autoimmune disease had more elevated serum immunoglobulins, and more effector memory T cells than those without autoimmunity.
Evidence of autoimmunity, including autoimmune diseases, is more prevalent in ICL than the general population, and should be considered part of this syndrome.
描述特发性 CD4 淋巴细胞减少症（ICL）患者的自身抗体和自身免疫性疾病。ICL 是一种罕见的免疫缺陷，其特征是在没有 HIV 或 HTLV 感染的情况下，绝对 CD4 T 计数<300 个/μl。
对 67 例患者进行了为期 11 年的单中心前瞻性研究。对患者进行了系统的风湿学评估和自身抗体检测，同时对部分患者进行了免疫细胞亚群的流式细胞术检测。
54%的患者有临床自身免疫证据，其中 34%至少有一种自身免疫性疾病，最常见的是自身免疫性甲状腺疾病。19%的患者有自身抗体或不完全的自身免疫性疾病特征。有自身免疫性疾病的患者血清免疫球蛋白水平更高，效应记忆 T 细胞更多。
与一般人群相比，ICL 患者中自身免疫证据，包括自身免疫性疾病更为普遍，应将其视为该综合征的一部分。
