北京大学学报（医学版）2023，Vol.55Issue(6)：966-974,9.DOI:10.19723/j.issn.1671-167X.2023.06.003
系统性红斑狼疮及成人Still病合并巨噬细胞活化综合征的临床特点及诊断指标
Clinical characteristics and diagnostic indicators of macrophage activation syndrome in patients with systemic lupus erythematosus and adult-onset Still's disease
摘要
Abstract
Objective:To analyze and compare the clinical and laboratory characteristics of macro-phage activation syndrome(MAS)in patients with systemic lupus erythematosus(SLE)and adult-onset Still's disease(AOSD),and to evaluate the applicability of the 2016 European League Against Rheuma-tism/American College of Rheumatology/Paediatric Rheumatology International Trials Organization classi-fication criteria for MAS complicating systemic juvenile idiopathic arthritis(sJIA)in different auto-immune diseases contexts and to propose new diagnostic predictive indicators.Methods:A retrospective analysis was conducted on the clinical and laboratory data of 24 SLE patients with MAS(SLE-MAS)and 24 AOSD patients with MAS(AOSD-MAS)who were hospitalized at Peking University People's Hospital between 2000 and 2018.Age-and sex-matched SLE(50 patients)and AOSD(50 patients)diagnosed in the same period without MAS episodes were selected as controls.The cutoff values for laboratory indi-cators predicting SLE-MAS and AOSD-MAS were determined using receiver operating characteristic(ROC)curves.Furthermore,the laboratory diagnostic predictive values for AOSD-MAS were used to im-prove the classification criteria for systemic juvenile idiopathic arthritis-associated MAS(sJIA-MAS),and the applicability of the revised criteria for AOSD-MAS was explored.Results:Approximately 60％of SLE-MAS and 40％of AOSD-MAS occurred within three months after the diagnosis of the underlying diseases.The most frequent clinical feature was fever.In addition to the indicators mentioned in the diag-nosis criteria for hemophagocytic syndrome revised by the International Society for Stem Cell Research,the MAS patients also exhibited significantly elevated levels of aspartate aminotransferase and lactate de-hydrogenase,along with a significant decrease in albumin.Hemophagocytosis was observed in only about half of the MAS patients.ROC curve analysis demonstrated that the optimal discriminative values for diagnosing MAS was achieved when SLE patients had ferritin level ≥1 010 μg/L and lactate dehydroge-nase levels≥359 U/L,while AOSD patients had fibrinogen levels≤225.5 mg/dL and triglyceride levels≥2.0 mmol/L.Applying the 2016 sJIA-MAS classification criteria to AOSD-MAS yielded a diagnostic sensitivity of 100％and specificity of 62％.By replacing the less specific markers ferritin and fibrinogen in the 2016 sJIA-MAS classification criteria with new cutoff values,the revised criteria for classifying AOSD-MAS had a notable increased specificity of 86％.Conclusion:Secondary MAS commonly occurs in the early stages following the diagnosis of SLE and AOSD.There are notable variations in laboratory indica-tors among different underlying diseases,which may lead to misdiagnosis or missed diagnosis when using uniform classification criteria for MAS.The 2016 sJIA-MAS classification criteria exhibit high sensitivity but low specificity in diagnosing AOSD-MAS.Modification of the criteria can enhance its specificity.关键词
巨噬细胞活化综合征/噬血细胞性淋巴组织细胞增多症/系统性红斑狼疮/成人Still病/诊断Key words
Macrophage activation syndrome/Hemophagocytic lymphohistiocytosis/Systemic lupus erythematosus/Adult-onset Still's disease/Diagnosis分类
医药卫生引用本文复制引用
姚海红,杨帆,唐素玫,张霞,何菁,贾园..系统性红斑狼疮及成人Still病合并巨噬细胞活化综合征的临床特点及诊断指标[J].北京大学学报（医学版）,2023,55(6):966-974,9.基金项目
国家自然科学基金(81801618)Supported by the National Natural Science Foundation of China(81801618) （81801618）
