Hemophagocytic lymphohistiocytosis (HLH) is a life‐threatening, hyperinflammatory disorder, characterized by multiorgan failure, fever and cytopenias. The diagnosis of HLH and its subtype Macrophage Activation Syndrome (MAS) remains a challenge. Interleukin 18 (IL‐18) is emerging as a potential biomarker for HLH/MAS but is currently not a part of diagnostic criteria. This systematic review aimed to assess the potential role of IL‐18 in the diagnosis and monitoring of HLH and MAS, and was performed according to the Preferred Reporting Items for Systematic Reviews and Meta‐Analyses (PRISMA) guidelines. PubMed and Embase were searched on 30 January 2020. Studies included all subtypes of HLH and a range of underlying disorders in both children and adults. A total of 14 studies were included. Generally, serum IL‐18 was elevated in both primary and secondary HLH (> 1000 pg/ml) compared with other inflammatory conditions and with healthy individuals; thus, serum IL‐18 may be able to discriminate between HLH and other inflammatory conditions. Significantly increased IL‐18 (> 10 000 pg/ml) was also consistently described in MAS compared with other subtypes of HLH. The ability of IL‐18 to distinguish MAS from systemic juvenile idiopathic arthritis (JIA) is less unambiguous, as IL‐18 levels > 100 000 pg/ml were described in sJIA patients both with and without MAS. IL‐18 may help to differentiate between HLH subtypes and other inflammatory conditions. As HLH and MAS are rare disorders, only few and relatively small studies exist on the subject. Larger, prospective multi‐center studies are called for to assess the diagnostic precision of IL‐18 for HLH and MAS.

中文翻译：

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白细胞介素-18在噬血细胞性淋巴组织细胞增多症/巨噬细胞活化综合征诊断和监测中的作用——系统评价

噬血细胞性淋巴组织细胞增多症（HLH）是一种危及生命的高炎症性疾病，以多器官衰竭、发热和血细胞减少为特征。HLH 及其亚型巨噬细胞激活综合征 (MAS) 的诊断仍然是一个挑战。白细胞介素 18 (IL-18) 正在成为 HLH/MAS 的潜在生物标志物，但目前还不是诊断标准的一部分。本系统评价旨在评估 IL-18 在诊断和监测 HLH 和 MAS 中的潜在作用，并根据系统评价和荟萃分析的首选报告项目 (PRISMA) 指南进行。PubMed 和 Embase 于 2020 年 1 月 30 日进行了检索。研究包括 HLH 的所有亚型以及儿童和成人的一系列潜在疾病。共纳入 14 项研究。一般来说，与其他炎症性疾病和健康个体相比，原发性和继发性 HLH (> 1000 pg/ml) 的血清 IL-18 升高；因此，血清 IL-18 可能能够区分 HLH 和其他炎症状况。与其他 HLH 亚型相比，在 MAS 中也一致地描述了显着增加的 IL-18（> 10 000 pg/ml）。IL-18 区分 MAS 和全身性幼年特发性关节炎 (JIA) 的能力不太明确，因为在有和没有 MAS 的 sJIA 患者中，IL-18 水平 > 100 000 pg/ml。IL-18 可能有助于区分 HLH 亚型和其他炎症状况。由于 HLH 和 MAS 是罕见的疾病，因此关于该主题的研究很少且相对较小。更大，