幼年皮肌炎120例临床特点及治疗随访分析

Clinical Features and Therapeutic Analysis of Juvenile Dermatomyositis in 120 Children

目的研究幼年皮肌炎（JDM）的临床特征、治疗效果以及转归。方法回顾性分析2003年12月-2011年3月在北京儿童医院住院JDM患儿120例,分析其起病情况、临床表现、实验室检查及辅助检查、治疗方法、随访和预后。结果 120例患儿男55例,女65例;发病年龄1~14岁,平均年龄7岁。患儿均有典型的皮损及不同程度的肌肉症状,83例（69%）患儿有内脏受累,最常受累系统为呼吸系统（48%）。所有患儿肌酶增高,肌电图均表现为肌源性损害。120例患儿均采用糖皮质激素治疗,均在早期加用甲氨蝶呤,有肺损害及重症患儿加用环孢素或环磷酰胺。早期治疗效果及远期预后均较好,120例在急性期死亡7例,死于肺部受累并感染致呼吸衰竭5例,并巨噬细胞活化综合征2例。结论 JDM是一种少见疾病,以肌无力和皮肤损害为突出表现,其皮损具有特征性,各脏器功能评估对诊断和判断疾病严重性非常有益;糖皮质激素联合免疫抑制剂治疗JDM安全有效,且预后较好。

Objective To study the clinical features,therapeutic efficacy and prognosis of juvenile dermatomyositis（JDM）.Methods One hundred and twenty cases of JDM hospitalized in Beijing Children′s Hospital from Dec.2003 to Mar.2011 were collected.The clinical features,laboratory data,treatment and outcome of patients with JDM were analyzed.Results All the patients had typical skin manifestations and myositis with various extents.The male-to-female ratio was 1：1.2.The mean age at onset of symptoms was 7 years old（range from 1 to 14 years）.There were 83（69%） patients with internal organs involvement.Respiration system was the most common system involved（48%）.Muscle enzymes elevated in all of the patients.All of the patients had typical findings from electromyography.All of the patients were treated by steroids plus methotrexate in the early time of the course of disease.Using sislosporin or cyclophosphamide for severe or pulmonary da-maged patients had got successful result.The patients got better outcome.There were 5 patients died from respiratory failure and 2 patients died from macrophage activation syndrome.The long term complications were rare.Conclusions JDM is characterized by the muscle weakness and rash,the evaluation of the function of various organs are very useful to diagnose and determine the severity of the disease.The treatment using corticosteroid combined with immunosuppressive agents is safe and effective in the JDM.The outcome of the JDM is fairly good.