幼年型皮肌炎42例临床回顾分析

Clinical retrospective analysis of 42 cases of juvenile dermatomyositis.

目的 探讨幼年型皮肌炎的临床特点。方法 对 4 2例幼年型皮肌炎进行临床分析。结果 首发症状中单纯皮疹占 33 3% ,皮疹伴肌炎占 4 0 5 % ,单纯肌炎占 16 7%。后期钙质沉着占 2 6 2 %。实验室检查乳酸脱氢酶 (LDH)、天冬氨酸转氨酶 (AST)、磷酸肌酸激酶 (CPK)升高分别为 94 7%、83 4 %、6 2 2 % ,类风湿因子 (RF)和抗核抗体 (ANA)阳性率分别为 37 5 %、2 5 0 %。内脏损害中心脏、肺、胃肠道分别占 5 9 5 %、5 7 1%、19 1% ,无一例伴发恶性肿瘤。肌电图显示肌源性改变占 91 7% ,肌活检异常占 91 7%。结论 幼年型皮肌炎以皮肤损害为首发症状 ,后期可引起广泛钙质沉着 ;实验室检查中肌酶升高频率LDH >AST >CPK ;RF阳性率高于ANA ;内脏损害中以心脏和肺损害多见。

Objective To investigate the clinical characteristics of juvenile dermatomyositis (JDM).Methods The clinical manifestations of 42 JDM patients were analyzed.Results In the first manifestations of the disease,only rash was present in 33 3% of the patients,rash and dermatitis simutaneously in 40 5%,only dermatitis in 16 7%.Calcinosis late in the course of the disease was present in 26 2 %.In the laboratory data,the positive rate lactic dehydrogenase (LDH) was 94 7%,aspartate aminotransferase (AST) 83 4% and creatine phosphate kinase (CPK) 62 2% respectively,the positive rates of rheumatoid factor(RF) and antinuclear antibody(ANA) were 37 5% and 25 0% respectively.Organs involved in JDM were heart 59 5%;lungs 57 1%;gastrointestinal tract 19 1%.No one was complicated with malignancy.Electromyogram revealed myogenic damage(91 7%).The abnormality rate of muscle biopsy was 91 7%.Conclusion The first manifestation of juvenile dermatomyositis (JDM) which can result in calcinosis late in the course of the disease is cutaneous disease.The frequency of elevation of serum levels of the muscle enzymes is LDH>AST>CPK.The positive rate of rheumatoid factor(RF) is higher than that of antinuclear antibody (ANA).The cardiac and pulmonary involvement are often seen in the children of visceral abnormality.