全身型幼年特发性关节炎合并巨噬细胞活化综合征分类标准在成人斯蒂尔病中的应用探讨  

Application of the 2016 European League Against Rheumatism/American College of Rheumatology/ Pediatric Rheumatology International Trials Organization classification criteria for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis in adult-onset Still disease

作　　者：王然[1] 李挺[1] 叶霜[1] 扶琼[1] 鲍春德[1] Wang Ran;Li Ting;Ye Shuang;Fu Qiong;Bao Chunde(Department of Rheumatology,Renji Hospital,Shanghai Jiaotong University School of Medicine,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,200001

出　　处：《中华风湿病学杂志》2018年第8期508-515,共8页Chinese Journal of Rheumatology

基　　金：国家自然科学基金（81471596）;上海市重中之重临床医学中心建设项目（2017ZZ01024-002）

摘　　要：目的2016年EULAR／ACRHL童风湿病国际试验组织（PRITO）联合提出了全身型幼年特发性关节炎（sJIA）合并巨噬细胞活化综合征（MAS）新的分类标准，由于AOSD和sJIA的发病机制和临床表现类似，本研究旨在了解这一分类标准在评估AOSD合并MAS的临床价值。方法本研究采集了169例在上海交通大学医学院附属仁济医院住院治疗的AOSD患者。使用2016年sJIA合并MAS分类标准将AOSD患者分为AOSD合并MAS及AOSD无MAS组，使用χ2检验、Mann-WhitneyU检验、二元Logistic分析比较2组患者之间的临床特点差异，使用Kaplan-Meier和COX回归分析影响2组患者预后的因素。结果根据sJIA合并MAS分类标准，169例AOSD患者中共有56例（33．1％）合并MAS。AOSD合并MAS患者中脾脏肿大及心包炎/心肌炎的发生率高于AOSD无MAS的患者[42．9％与14．2％，OR值（95％CI）为4．50（2．13，9．51），P〈0．01；10．7％与0．9％，OR值（95％C1）为13．21（1．56，113．57），P〈0．01]，肝损害的发生率更高[67．8％与11．5％，OR值（95％CI）为0．18（7．26，36．33），P〈0．01]。AOSD合并MAS患者中有62．5％（35／56）接受了大剂量激素，5．4％（3／56）接受了激素冲击治疗，48．2％（27／56）使用了静脉丙种球蛋白，26．8％（15／56）使用了钙调磷酸酶抑制剂。AOSD合并MAS患者病死率高达8．9％（5／56），显著高于AOSD无MAS患者的1．8％（2／113）（OR=5．44，P〈0．05），满足sJIA合并MAS分类标准提示预后不良（OR=5．44，P=0．041），且血小板计数≤181×10^9／L（OR=12．17，P=-0．002），ALT〉48UIL（OR=9．43，P=-0．040）高度提示AOSD患者预后不良。结论sJIA合并MAS分类标准有助于早期识别危重AOSD患者，提示MAS诊断，而且简便易行，适于临床使用。AOSD患者中符合sJIA合并MAS分类标准的患者病情更加严重，需要更大剂量的糖皮质激素和更积极的免疫抑制Objective In 2016, European League Against Rheumatism （EULAR）/American College of Rheumatology （ACR）/Pediatric rheumatology international trials organization （PRINTO） released the classification criteria for macrophage activation syndrome （MAS） in patients ,with systemic juvenile idiopathic arthritis （sJIA）. Due to the similarities of both clinical manifestations and pathogenesis between adult-onset Still dsease （AOSD） and sJIA, we hope to evaluate the 2016 sJIA-AMS classification in AOSD patients. Methods A total of 169 AOSD patients who were hospitalized in Renji Hospital were enrolled in this study. AOSD patients were divided into AOSD with MAS and AOSD without MAS, using the 2016 sJIA-MAS criteria.The data of the two groups were analyzed by Chi-square test, Mann-Whitney U test and binary Logistic analysis, and factors influencing the prognosis of patients were analyzed by Kaplan-Meier and COX regression analysis. Results According to sJIA-MAS criteria, 56 AOSD patients with MAS were identified in all the 169 AOSD cases. In AOSD patients, the incidence of splenomegaly and pericarditis/myocarditis was significantly higher in patients with MAS than in AOSD without MAS [42.9% vs 14.2%, 0R（95%CI）=4.50（2.13, 9.51）, P〈0.01; 10,7% vs 0.9%, 0R（95%CI）=13.21（1.56, 113.57）, P〈0.01], also the incidence of liver dysfunction was higher in AOSD with MAS [67.8% vs 11.5%, 0R（95%CI）=0.18（7.26, 36.33）, P〈0.01]. Among the AOSD with MAS, 62.5% （35/56） of these patients received large-dose glucocorticoid therapy, 5.4% （3/56） received the glucocorticoid pulse therapy, 48.2%（27/56） were treated with IVIG, and 26.8%（15/56） were treated with calcium phosphatase inhibitors. The mortality rates of AOSD with MAS was 8~9%（5/56）, which was significantly higher than 1.8%（2/113） （0R=5.44, P〈0.05）, the mortality rate of the AOSD without MAS. Patients who fulfilled the sJIA-MAS criteria suggested poor prognosis （OR=0.041, P=5.44）, and the platelet count ≤

关 键 词：斯蒂尔病 成年型 巨噬细胞活化 关节炎 幼年型类风湿 诊断 

分 类 号：R593.2[医药卫生—内科学]