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在患有全身性红斑狼的患者中,区分通过莱克或经典补充途径激活

Mads Lamm Larsen^1,2, Anne Troldborg^1,2,3, Erik J M Toonen⁴

¹Department of Biomedicine, Aarhus University, Aarhus, Denmark.

Clinical and experimental immunology

|July 5, 2023

概括

通过古典途径 (CP) 激活补充剂与活跃的系统性红性狼 (SLE) 有关,而乳素途径 (LP) 可能与狼性炎 (LN) 特定. 这项研究区分了SLE患者的CP和LP激活.

科学领域:

免疫学免疫学免疫学
类风湿病学类风湿病学
补充系统补充系统

背景情况:

补充激活是系统性红斑狼 (SLE) 病原体的核心.
目前的临床实践缺乏测试来监测特定的补充通路激活 (古典 (CP),莱克 (LP),替代 (AP)).
区分CP和LP激活对于理解SLE和开发向疗法至关重要.

研究的目的:

开发和验证新型ELISA来量化CP特异性 (C1s/C1抑制剂) 和LP特异性 (MASP-1/C1抑制剂) 激活复合体.
在SLE患者中调查特定途径补充激活和疾病活性 (SLEDAI) 与狼性炎 (LN) 之间的关联.

主要方法:

使用新开发的ELISA对156名SLE患者和50名对照患者的C1s/C1抑制剂和MASP-1/C1抑制剂复合物的量化.
根据使用SLEDAI评分 (≥6为活性,<6为低活性) 的疾病活动来对SLE患者进行分层.
活跃的SLE,不活跃的SLE,活跃的LN和非活跃的LN群体之间的补充激活标志物的比较.

主要成果:

在活跃的SLE患者中,C1s/C1抑制剂复合体显著升高 (SLEDAI ≥6) 并与疾病活性相关 (r = .29,P < 0.01).
与非活性LN (P = 0.02) 相比,MASP-1/C1抑制剂复合物在活性狼性炎 (LN) 中显著增加.
对于MASP-1/C1抑制剂与整体SLE疾病活性没有发现显著的差异或相关性.

结论:

经典途径 (CP) 在活跃的SLE中被激活,由升高的C1s/C1抑制剂表明.
通过MASP-1/C1抑制剂测量的莱克通路 (LP) 激活可能与狼性炎 (LN) 特别相关.
这些发现支持进一步研究针对特定途径的补充激活,以定制SLE治疗策略.

关键词:

这是一种C1抑制剂.经典的路径是古典的路径.互补系统是互补的系统.莱克通路的路径系统性红血性狼 (Systemic Lupus Erythematosus) 是一种全身性狼.

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