受体相互作用蛋白激酶与成人斯蒂尔病病情活动的相关性  

Association of receptor-interacting serine/threonine-protein kinase 1/3 with disease activity of adult-onset Still's disease

作　　者：林镇[1] 郭茹茹[1] 陈晓翔[1] 余跃天[1,2] 吕良敬[1] LIN Zhen;GUO Ruru;CHEN Xiaoxiang;YU Yuetian;LU Liangjing(Department of Rheumatology;Department of Critical Care Medicinez Renji Hospitalj School of Medicinej Shanghai ]iaotong University, Shanghai 200001, China)

机构地区：[1]上海交通大学医学院附属仁济医院风湿科,上海200001 [2]上海交通大学医学院附属仁济医院重症医学科,上海200001

出　　处：《临床与病理杂志》2018年第5期966-971,共6页Journal of Clinical and Pathological Research

基　　金：国家重点研发计划(2017YFC0909000);国家自然科学基金(81373209);上海市教育委员会高峰高原学科建设计划(20152214)~~

摘　　要：目的:研究成人斯蒂尔病(adult-onset Still’s disease,AOSD)患者外周血细胞内受体相互作用蛋白激酶-1/3(receptor-interacting serine/threonine-protein kinase 1/3,RIPK1/3)的表达水平,分析RIPK1/3与AOSD病情活动的相关性,并初步探讨其在疾病中的作用机制。方法:回顾性分析2010年6月至2015年6月就诊于我院风湿科58例AOSD患者的病史信息,明确与疾病活动相关的临床指标。为初步探讨其中潜在的机制,采用流式细胞术检测2016年9月至2017年12月17例AOSD住院患者及35例健康对照外周血中性粒细胞和单核细胞内RIPK1/3的表达水平。结果:58例AOSD患者中性粒细胞绝对值和单核细胞绝对值与病情严重程度(系统评分)显著相关(r=0.6157,r=0.4278,P<0.001)。初步机制探讨中发现17例患者外周血中性粒细胞和单核细胞IPK1/3的表达水平显著低于健康对照(P<0.0001),且患者外周血单核细胞中高表达RIPK1和RIPK3的单核细胞比例与血沉呈负相关(r=.0.5332,P<0.05;r=.0.5098,P<0.05)。结论:中性粒细胞及单核细胞绝对计数可作为AOSD病情活动性良好的监测指标,且RIPK1/3在单核细胞中的表达情况可能在致病中发挥重要作用。Objective： To investigate the receptor-interacting serine/threonine-protein kinase 1/3（RIPK1/3） levels in peripheral blood cells of patients with adult-onset Still＇s disease（AOSD）, and analyze its relationship with the disease activity. The role of RIPK1/3 in the pathogenesis of AOSD was also explored. Methods： Patients diagnosed with AOSD between June 2010 and June 2015 in Renji Hospital were screened and analyzed in thisretrospective cohort study, potential markers which might relate to the activity of the disease were also tested. To explore and confirm the mechanism underlying the markers, intercellular RIPK1/3 productions by peripheral blood cells were detected by flow cytometry between September 2016 and December 2017. Results： The absolute counts of neutrophils and monocytes in patients were correlated with disease severity（r=0.6157, r=0.4278,P〈0.001）. Intercellular RIPK1/3 levels by neutrophils and monocytes were significantly lower in AOSD than those in healthy controls（P〈0.0001）. The percentage of monocytes with low RIPK1/3 in patients were correlated with disease severity（P〈0.05）. Conclusion： The absolute counts of neutrophils and monocytes in AOSD can be good markers to reflect the disease activity and RIPK1/3 levels in monocytes may possibly play an important role in the pathogenesis of AOSD.

关 键 词：成人STILL病 受体相互作用蛋白激酶-1/3 疾病活动性 

分 类 号：R593.2[医药卫生—内科学]