Primary immune regulatory disorders (PIRDs) constitute a spectrum of inborn errors of immunity (IEIs) that are primarily characterized by autoimmunity, lymphoproliferation, atopy, and malignancy. In PIRDs, infections are infrequent compared to other IEIs. While susceptibility to infection primarily stems from antibody deficiency, it is sometimes associated with additional innate immune and T or NK cell defects. The use of immunotherapy and chemotherapy further complicates the immune landscape, increasing the risk of diverse infections. Recurrent sinopulmonary infections, particularly bacterial infections such as those associated with staphylococcal and streptococcal organisms, are the most reported infectious manifestations. Predisposition to viral infections, especially Epstein–Barr virus (EBV)-inducing lymphoproliferation and malignancy, is also seen. Notably, mycobacterial and invasive fungal infections are rarely documented in these disorders. Knowledge about the spectrum of infections in these disorders would prevent diagnostic delays and prevent organ damage. This review delves into the infection profile specific to autoimmune lymphoproliferative syndrome (ALPS), Tregopathies, and syndromes with autoimmunity within the broader context of PIRD. Despite the critical importance of understanding the infectious aspects of these disorders, there remains a scarcity of comprehensive reports on this subject.

中文翻译：

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免疫调节障碍中的感染

原发性免疫调节障碍 (PIRD) 构成一系列先天性免疫错误 (IEI)，其主要特征为自身免疫、淋巴细胞增殖、特应性和恶性肿瘤。与其他 IEI 相比，PIRD 中的感染并不常见。虽然对感染的易感性主要源于抗体缺乏，但有时也与其他先天免疫和 T 或 NK 细胞缺陷有关。免疫疗法和化疗的使用使免疫环境进一步复杂化，增加了多种感染的风险。复发性鼻窦肺部感染，特别是细菌感染，例如与葡萄球菌和链球菌相关的细菌感染，是报道最多的感染表现。还观察到病毒感染的倾向，尤其是 Epstein-Barr 病毒 (EBV) 诱导的淋巴细胞增殖和恶性肿瘤。值得注意的是，在这些疾病中很少有分枝杆菌和侵袭性真菌感染的记录。了解这些疾病的感染谱可以防止诊断延误并防止器官损伤。本综述深入研究了自身免疫性淋巴增殖综合征 (ALPS)、Tregopathies 以及 PIRD 更广泛背景下的自身免疫综合征的特定感染特征。尽管了解这些疾病的传染性方面至关重要，但仍然缺乏关于这一主题的综合报告。