Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic Arthritis

Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic Arthritis
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DOI:
10.1002/acr.23277
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发表时间:
2018-03-01
影响因子:
4.7
通讯作者:
Grom, Alexei A.
Grom, Alexei A.
中科院分区:
医学2区
文献类型:
--
作者:
Schulert, Grant S.;Minoia, Francesca;Grom, Alexei A.

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目的评估2016年巨噬细胞活化综合征(MAS)分类标准的性能与系统性幼年特发性关节炎(JIA)谁开发MAS,而治疗biologicalmedicines.MethodsA系统性文献综述进行识别与MAS患者,而正在与白细胞介素(IL)-1和IL-6阻断剂治疗。临床和实验室信息进行了比较,以前编制的历史cohol.ResultsEighteen出版物确定,并删除重复后,35例canakinumab和49例托珠单抗治疗的患者可用于分析; 5阿那白滞素治疗的患者被排除在外,由于数量有限。与历史队列或卡那单抗治疗患者相比,MAS分类标准将托西珠单抗治疗患者分类为MAS的可能性较小(分别为56.7%、78.5%和84%; P < 0.01)。在接受canakinumab治疗期间发生MAS的患者在MAS发作时的铁蛋白倾向于低于历史队列(4,050 vs 5,353 ng/ml; P = 0.18),但在其他主要临床或实验室特征方面没有差异。相比之下,托珠单抗治疗期间发生MAS的患者不太可能发热,铁蛋白水平明显较低(1,152 vs 5,353 ng/ml; P < 0.001)。在接受托珠单抗治疗的患者中,MAS的其他特征更为明显,包括血小板计数降低、纤维蛋白原降低和天冬氨酸转氨酶水平升高。与托珠单抗或canakinumab治疗的MAS患者的死亡率没有显着不同的历史coherent.ConclusionThese研究结果显示MAS功能的实质性改变,可能会限制效用的定义标准诊断的系统性JIA患者用生物制剂治疗。
ObjectiveTo assess performance of the 2016 macrophage activation syndrome (MAS) classification criteria for patients with systemic juvenile idiopathic arthritis (JIA) who develop MAS while treated with biologic medications.MethodsA systematic literature review was performed to identify patients with MAS while being treated with interleukin (IL)-1 and IL-6 blocking agents. Clinical and laboratory information was compared to a large previously compiled historical cohort.ResultsEighteen publications were identified, and after removing duplicates, 35 patients treated with canakinumab and 49 patients with tocilizumab were available for analysis; 5 anakinra-treated patients were excluded due to limited numbers. MAS classification criteria were less likely to classify tocilizumab-treated patients as having MAS compared to the historical cohort or canakinumab-treated patients (56.7%, 78.5%, and 84%, respectively; P < 0.01). Patients who developed MAS while treated with canakinumab trended towards lower ferritin at MAS onset than the historical cohort (4,050 versus 5,353 ng/ml; P = 0.18) but had no differences in other cardinal clinical or laboratory features. In comparison, patients who developed MAS while treated with tocilizumab were less likely febrile and had notably lower ferritin levels (1,152 versus 5,353 ng/ml; P < 0.001). Other features of MAS were more pronounced in patients treated with tocilizumab, including lower platelet counts, lower fibrinogen, and higher aspartate aminotransferase levels. Mortality rates for patients with MAS treated with tocilizumab or canakinumab were not significantly different from the historical cohort.ConclusionThese findings show substantial alterations in MAS features that may limit utility of defined criteria for diagnosis of systemic JIA patients treated with biologic agents.