血清IgG水平和IgG4相关性疾病反应指数在IgG4-RD活动性评估中的应用  

The application of serum IgG and IgG4-related disease responder index in the disease activity assessment of IgG4-RD

作　　者：纪宗斐 马玲瑛[1,2,3] 张丽娟 马莉莉 陈慧勇[1,2,3] 姜林娣 JI Zong-fei;MA Ling-ying;ZHANG Li-juan;MA Li-li;CHEN Hui-yong;JIANG Lin-di(Department of Rheumatology,Zhongshan Hospital,Fudan University,Shanghai 200032,China;Evidence-Based Medicine Center,Fudan University,Shanghai 200032,China;Institute of Rheumatology,Immunology and Allergy,Fudan University,Shanghai 200032,China)

机构地区：[1]复旦大学附属中山医院风湿免疫科,上海200032 [2]复旦大学循证医学中心,上海200032 [3]复旦大学风湿免疫过敏中心,上海200032

出　　处：《复旦学报（医学版）》2020年第5期647-653,共7页Fudan University Journal of Medical Sciences

基　　金：上海市浦江风湿青年医师培育计划(SPROG201801)。

摘　　要：目的验证IgG4相关性疾病(IgG4-related disease,IgG4-RD)反应指数(responder index,RI)在IgG4-RD疾病活动性评估中的诊断效率,并进一步探索新的生物学标志物。方法纳入117例符合2012年日本标准的IgG4-RD患者,由3名专科专家通过医师整体评估(physician global assessment,PGA)将患者分为活动组(n=78)和稳定组(n=39)。总结患者的一般情况、临床资料、实验室检查和IgG4-RD RI,分析两组患者一般情况和实验室检查的差异。进一步分析血清标志物及IgG4-RD RI评估IgG4-RD疾病活动性的敏感度、特异度、阳性预测值、阴性预测值和曲线下面积。结果活动组IgG4-RD患者的外周血嗜酸性粒细胞百分比、血小板、血沉、C反应蛋白、IgG、IgG4、球蛋白、白介素6(interleukin-6,IL-6)和可溶性IL-2受体(soluble IL-2 receptor,sIL2R)较稳定组显著升高;血红蛋白、总胆固醇和甘油三酯显著降低。IgG、IgG4、球蛋白、血沉和sIL-2R水平均与IgG4-RD RI呈正相关(P<0.05)。IgG4-RD RI和血清IgG水平用于判断疾病活动性的曲线下面积达到0.940和0.916。以IgG4-RD RI≥6为标准,敏感度和特异度分别可达到92.3%和86.5%;以IgG≥11.6 g/L为标准,敏感度和特异度为89.1%和82.6%。结论在IgG4-RD活动性评估中,IgG4-RD RI评分具有最高的诊断效率,血清IgG水平也可作为疾病活动性的标志物,敏感度和特异度较高,IgG4-RD RI评分和血清IgG水平是临床诊治中可靠、实用的评估方法。Objective To verify the diagnostic efficiency of the IgG4-related disease(IgG4-RD)responder index(RI)in the assessment of IgG4-RD disease activity,and to further explore new comprehensive clinical diagnostic criteria.Methods A total of 117 IgG4-RD patients who met the 2012 diagnosis criteria in Japan were divided into the active group(n=78)and stable group(n=39)by three experts using physician global assessment(PGA).We collected the general situation,clinical data,laboratory tests and IgG4-RD RI of the patients.The differences between the general conditions and laboratory tests of the two groups were analyzed.The serum markers and IgG4-RD RI were further analyzed to evaluate the sensitivity,specificity,positive predictive value,negative predictive value,and area under the curve of IgG4-RD disease activity.Results Peripheral blood eosnophils percentage(E%),platelet(PLT),erythrocyte sedimentation rate(ESR),C-reactive protein(CRP),IgG,IgG4,globulin,interleukin-6(IL-6)and soluble IL-2 receptor(sIL2R)in active group were significantly higher than those in the stable group;while hemoglobin,total cholesterol,and triglycerides were significantly reduced.IgG,IgG4,globulin,ESR and sIL-2R levels were positively correlated with IgG4-RD RI(P<0.05).In the assessement of disease activity,the area under the curve of IgG4-RD RI and serum IgG levels were 0.940 and 0.916,respectively.The sensitivity and specificity were up to 92.3%and 86.5%,respectively for IgG4-RD RI≥6.The sensitivity and specificity were 89.1%and 82.6%for IgG≥11.6 g/L.Conclusion In the assessment of IgG4-RD disease activity,the IgG4-RD RI exhibited the highest diagnostic efficiency.Serum IgG level can also be used as a marker of disease activity,with high sensitivity and specificity.Both IgG4-RD RI and IgG were reliable and practical methods in clinical disease assessment.

关 键 词：IgG4相关性疾病(IgG4-RD) IGG4 IgG 球蛋白 反应指数(RI) 

分 类 号：R593.2[医药卫生—内科学]