成人系统性自身炎症性疾病在发热待查诊治中的研究进展

doi:10.3877/cma.j.issn.1674-6880.2023.06.012

不明原因发热（fever of unknown-origins，FUO）是临床上十分常见的疾病，但对诊断FUO的具体准确标准仍未有定论^[1]。在1998年中国发热性疾病学术研讨会上，FUO被定义为：发热持续2 ~ 3周及以上，体温≥ 38.5 ℃，经详细询问病史、体格检查和常规实验室检查仍不能明确诊断者^[2]。由于病因多样、临床症状不典型、特异性实验室检查或影像学检查的缺乏，FUO的诊断和治疗有诸多困难。对于该病的延迟诊断会导致病情迁延，如病因诊断不明确，则会导致药物错用或经验性用药，延误治疗乃至加重病情、诱发致命性的并发症。目前认为，FUO的病因可以分为以下几种：经典FUO（感染、癌症、自身免疫和自身炎症性疾病以及其他原因）；医院FUO；免疫缺陷相关的FUO和旅行相关的FUO^[1]。其中相似而又不同的两类疾病——自身炎症性疾病和自身免疫性疾病占FUO病例的5% ~ 32%^[1]。成人系统性自身炎症性疾病（systemic auto-inflammatory diseases，SAIDs）是一类较为少见的先天性免疫疾病，伴有白细胞介素1β（interleukin-1beta，IL-1β）反应、IL-18反应或两者失调，往往具有周期性发热；而由于当前临床医生的了解度较低以及诊断的相关局限，诊断延迟可达7.3年^[3]，因此在FUO的病因诊断过程中及时考虑该病，对于早期明确诊断、优化治疗和提高患者生存质量有重要作用。

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