Macrophage activation-like syndrome: an immunological entity associated with rapid progression to death in sepsis.
Macrophage activation-like syndrome: an immunological entity associated with rapid progression to death in sepsis.
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DOI：
10.1186/s12916-017-0930-5
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发表时间：
2017-09-18
期刊：
影响因子：
9.3
通讯作者：
Hellenic Sepsis Study Group
中科院分区：
文献类型：
--
作者：
Kyriazopoulou E;Leventogiannis K;Norrby-Teglund A;Dimopoulos G;Pantazi A;Orfanos SE;Rovina N;Tsangaris I;Gkavogianni T;Botsa E;Chassiou E;Kotanidou A;Kontouli C;Chaloulis P;Velissaris D;Savva A;Cullberg JS;Akinosoglou K;Gogos C;Armaganidis A;Giamarellos-Bourboulis EJ;Hellenic Sepsis Study Group
一项随机临床试验的亚分析表明，巨噬细胞激活样综合征（MALS）患者的脓毒症生存受益于白细胞介素(IL)-1阻断。本研究旨在探讨肌萎缩侧索硬化症的发生频率，并寻找一种诊断和预后的生物标志物。感染和全身炎症反应综合征患者被分配到一个测试队列（n = 3417）和一个验证队列（n = 1704）。对于噬血细胞综合征评分阳性和/或同时伴有肝胆功能障碍和弥散性血管内凝血的患者，诊断为MALS。采用Logistic回归分析估计MALS对两组患者10天死亡率的预测价值。前24 h测定血铁蛋白、sCD163、IL-6、IL-10、IL-18、干扰素γ (IFN-γ)、肿瘤坏死因子α (TNF-α)；在第3天对747例患者重复铁蛋白测量。在试验组和验证组中，MALS的发生率分别为3.7%和4.3%。在这两个队列中，MALS是10天死亡率的独立危险因素。铁蛋白水平高于4420 ng/ml， 28 d后死亡率分别为66.7%和66%。铁蛋白水平高于4420 ng/ml与IL-6、IL-18、INF-γ和sCD163升高以及IL-10/TNF-α比值降低相关，表明促炎现象占主导地位。第3天铁蛋白下降低于15%与10天后不良结果的敏感性超过90%相关。在一个独立的瑞典队列（n = 109）中也证实了这种高死亡率风险。MALS是脓毒症中一个独立的危及生命的实体。铁蛋白测量可以提供肌萎缩侧索硬化症的早期诊断，并可能允许特定的治疗。
A subanalysis of a randomized clinical trial indicated sepsis survival benefit from interleukin (IL)-1 blockade in patients with features of the macrophage activation-like syndrome (MALS). This study aimed to investigate the frequency of MALS and to develop a biomarker of diagnosis and prognosis. Patients with infections and systemic inflammatory response syndrome were assigned to one test cohort (n = 3417) and a validation cohort (n = 1704). MALS was diagnosed for patients scoring positive either for the hemophagocytic syndrome score and/or having both hepatobiliary dysfunction and disseminated intravascular coagulation. Logistic regression analysis was used to estimate the predictive value of MALS for 10-day mortality in both cohorts. Ferritin, sCD163, IL-6, IL-10, IL-18, interferon gamma (IFN-γ), and tumor necrosis factor alpha (TNF-α) were measured in the blood the first 24 h; ferritin measurements were repeated in 747 patients on day 3. The frequency of MALS was 3.7% and 4.3% in the test and the validation cohort, respectively. In both cohorts, MALS was an independent risk factor for 10-day mortality. A ferritin level above 4420 ng/ml was accompanied by 66.7% and 66% mortality after 28 days, respectively. Ferritin levels above 4420 ng/ml were associated with an increase of IL-6, IL-18, INF-γ, and sCD163 and a decreased IL-10/TNF-α ratio, indicating predominance of pro-inflammatory phenomena. Any less than 15% decrease of ferritin on day 3 was associated with more than 90% sensitivity for unfavorable outcome after 10 days. This high mortality risk was also validated in an independent Swedish cohort (n = 109). MALS is an independent life-threatening entity in sepsis. Ferritin measurements can provide early diagnosis of MALS and may allow for specific treatment.
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影响因子：
8.8
作者：
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