Objective

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.

Methods

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.

Results

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.

Conclusions

Evidence of autoimmunity, including autoimmune diseases, is more prevalent in ICL than the general population, and should be considered part of this syndrome.

中文翻译：

---

特发性CD4淋巴细胞减少症患者的前瞻性队列中自身抗体，免疫表型和自身免疫性疾病的特征

目的

在特发性CD4淋巴细胞减少症（ICL）患者的前瞻性队列中表征自身抗体和自身免疫性疾病，这种罕见的免疫缺陷症的特征是在没有HIV或HTLV感染的情况下，绝对CD4 ⁺ T计数<300细胞/μl。

方法

在11年中对67位患者进行了单中心前瞻性研究。系统地进行了风湿病学评估和自身抗体的测定，并对一部分患者进行了免疫细胞亚群的流式细胞术。

结果

接受转诊的患者中有54％具有自身免疫性的临床证据，其中34％患有至少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％的患者具有自身抗体或自身免疫性疾病的特征不完整。与没有自身免疫的患者相比，具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。

结论

ICL中的自身免疫性疾病（包括自身免疫性疾病）证据比一般人群更为普遍，应视为该综合征的一部分。