儿童多发性肌炎和皮肌炎临床分析

Clinical analysis of children with polymyositis and dermatomyositis

目的探讨儿童多发性肌炎(PM)和皮肌炎(DM)的临床特点与治疗。方法分析1999年4月-2004年6月诊断为PM和DM6例患儿的临床特点、实验室检查、肌电图和肌肉活检结果,以及糖皮质激素联合免疫抑制剂治疗疗效和预后。结果PM和DM患儿均有对称性近端肌无力,皮肤改变为双上眼睑水肿性淡紫红色斑和Gottron征,部分患儿累及关节、心脏、肺脏和消化系统;6例患儿均有肌酶升高,肌电图呈肌源性损害,肌肉活检示肌炎改变;泼尼松联合免疫抑制剂治疗后,肌力恢复至Ⅱ～Ⅲ级约为1～3个月,肌力从Ⅲ～Ⅳ级恢复至正常为7～13个月,四肢肌力早于颈部肌力恢复;血清肌酶1～6个月恢复正常,ESR、CRP、肝功能异常治疗后均恢复正常;6例患儿中5例已停止治疗,最长停药时间为4年,肌力正常,无发热、皮疹等情况,1例治疗中出现对称性腕关节、近端指间关节肿痛,活动受限。结论PM和DM以肌无力和皮肤损害为突出表现,其他系统亦可受累,各脏器功能评估对诊断和判断疾病严重性非常有益;肌电图和肌肉病理检查是确诊PM和DM及与其他原因引起的肌病鉴别的重要依据;糖皮质激素联合免疫抑制剂治疗PM和DM安全有效,儿童PM和DM预后较好。

Objectives To analyze the clinical features and treatment of polymyositis （PM） and dermatomyositis （DM） in children. Methods The clinical characteristics, result of blood tests, electromyography （EMG） and muscles biopsy, the treatment using corticosteroid combined with immunosupressive agents and the outcome of 6 children diagnosed as having PM and DM from April, 1999 to June, 2004, were anlalysed. Results All 6 patients had symmetric proximal muscle weakness. The skin involvement consisted of the eyes surrounded by a violet discoloration with swelling and Gottron＇s sign. The joints, heart, lungs and digestive system were involved in some of them. The creatine kinase levels were elevated in all. The EMG showed myopathic injury pattern. The muscle biopsy showed inflammatory myopathy. After being treated by combining corticosteroid with immunosupressive agents, the patients＇ muscle power improved to 2 - 3 degree one to three months later, and from 3 - 4 degree to normal after seven to thirteen months. The muscle power of extremity muscles improved earlier than that of neck flexor muscles. It took one to six months for creatine kinase level to decrease to normal. Erythrocyte sedimentation rate, C-reaction protein and liver function were normal during treatment. Five patients had discontinued the treatment, one of them experienced the longest time without treatment for four years, and had normal muscle power and no fever and rash during follow up. Another one patient who presented arthralgia, symmetric joint swelling and limited joint function in the wrist joints and proximal interpbalangeal joints during treatment. Conclusions PM and DM were characterized by the muscle weakness and rash, however, muhiple systems could be involved. The evaluation of the function of various organs were very useful to diagnose and determine the severity of the disease. EMG and muscle biopsy were the definitive test not only for establishing the diagnosis of PM and DM but also for excluding the myopathy caused by other diseases. The treatment using corticosteroid combined with immunosupressive agents is safe and effective in the children with PM and DM. The outcome of the children with PM and DM is fairly good.