幼年型皮肌炎并发巨噬细胞活化综合征临床分析  

Juvenile dermatomyositis-associated macrophage activation syndrome:clinical analysis and literature review

作　　者：徐莉[1] 刘明月[1] 罗冲[1] 张宇[1] 汪利 王雅婷[1] 唐雪梅[1] XU Li;LIU Mingyue;LUO Chong;ZHANG Yu;WANG Li;WANG Yating;TANG Xuemei(Department of Rheumatology and Immunology,Children's Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorder,China International Science and Technology Cooperation base of Child development and Critical Disorders,National Clinical Research Center for Child Health and Disorders,Chongqing Key Laboratory of Child Infection and Immunity,Chongqing 400014,China)

机构地区：[1]重庆医科大学附属儿童医院风湿免疫科,儿童发育疾病研究教育部重点实验室,国家儿童健康与疾病临床医学研究中心,儿童发育重大疾病国家国际科技合作基地,儿童感染免疫重庆市重点实验室,重庆400014

出　　处：《临床皮肤科杂志》2023年第9期526-530,共5页Journal of Clinical Dermatology

摘　　要：目的:分析幼年型皮肌炎并发巨噬细胞活化综合征(JDM-MAS)的临床和实验室特点、治疗及转归。方法:回顾性分析我院收治的5例JDM-MAS患儿的临床资料,并进行文献复习。结果:5例JDM-MAS患儿中男3例,女2例,年龄4.83~14.92岁,均于首次就诊时发生MAS。5例均有皮疹及肌炎表现,均处于疾病活动期,首发表现均有发热,4例伴出血,1例伴神经系统受累表现。体格检查示5例患儿肝脏肿大,4例脾脏肿大,5例患儿均有不同程度的血细胞减少、丙氨酸氨基转移酶(ALT)和天冬氨酸氨基转移酶(AST)升高、铁蛋白升高、高甘油三酯血症及低纤维蛋白原血症,3例有组织噬血现象,2例肌炎特异性抗体阳性。2例合并间质性肺疾病。所有患儿确诊后均予静脉滴注甲泼尼龙冲击治疗,4例联合环孢素抑制免疫,1例加用甲氨蝶呤治疗,1例加用环磷酰胺抑制免疫,2例联合静脉滴注人免疫球蛋白抗炎。5例均符合2016欧洲抗风湿病联盟(EULAR)/美国风湿病学会(ACR)的sJIA-MAS诊断标准。5例患儿经过治疗均好转出院。文献复习共检索到14例JDM-MAS,9例符合2016EULAR/ACR的sJIA-MAS诊断标准。结论:当JDM合并持续发热、脾肿大、血细胞减少、高甘油三酯血症、铁蛋白升高、纤维蛋白原降低时需警惕发生MAS。JDM-MAS诊断可借鉴2016 EULAR/ACR制定的s JIA-MAS标准。JDM-MAS的总体预后较好,糖皮质激素联合环孢素是治疗JDM-MAS的首选药物。Objective:To analyze the clinical features,laboratory findings,treatment and prognosis of juvenile dermatomyositisassociated macrophage activation syndrome(JDM-MAS).Methods:A total of 5 JDM-MAS patients admitted to the Children's Hospital of Chongqing Medical University from January 2008 to June 2021 were retrospectively analyzed,and a total of 14 cases of JDM-MAS from literatures were reviewed.Results:There were 3 males and 2 females.The age range was 4.83 to 14.92 years old.All had MAS on the first visit.Five cases had rash and myositis.Five cases were in active disease condition.Five cases had fever,4 cases had bleeding,and 1 case had nervous system involvement at the onset.Physical examination showed 5 cases with hepatomegaly and 4 cases with splenomegaly.All patients had cytopenias,elevated alanine aminotransferase and aspertate aminotransferase,hyperferritinemia,hypertriglyceridemia and hypofibrinogenemia.Three cases had hemophagocyto sis in bone marrow.Myositis-specific antibodies were positive in 2 cases.Two cases were complicated with interstitial lung disease.All patients were treated with pulsed methylprednisolone.Other treatments included cyclosporine A(4 cases),methotrexate(1 case),cyclophosphamide(1 case)and intravenous immunoglobulin(2 cases).All patients met the 2016 EULAR/ACR diagnostic criteria for MAS complicating sJIA.All patients had a good prognosis.A total of 14 cases of JDM-MAS were retrieved from the literature and were reviewed.Nine cases met the 2016 EULAR/ACR diagnostic criteria for sJIA-MAS.Conclusion:MAS should be considered in patients with JDM present as persistent fever,splenomegaly,cytopenia,hypertriglyceridemia,hyperferritinemia and/or hypofibrinogenemia.The diagnosis of JDM-MAS can refer to 2016 EULAR/ACR sJIA-MAS diagnostic criteria.Most patients with JDM-MAS had a good prognosis.Glucocorticoid combined with cyclosporine is the preferred treatment forJDM-MAS.

关 键 词：巨噬细胞活化综合征 幼年型皮肌炎 诊断标准 全身型幼年特发性关节炎 

分 类 号：R593.2[医药卫生—内科学]