儿童特发性炎症性肌病1例
A Case with Idiopathic Inflammatory Myositis in Children

目的：提高对儿童特发性炎症性肌病的认识。方法：分析1例特发性炎症性肌病患儿的临床特点、病理改变及诊疗经过。结果：患儿，女，9岁，以横纹肌溶解起病，临床表现为双下肢肌痛、肌无力，血清肌酸激酶、肌红蛋白明显升高；肌电图提示为肌源性损害；肌活检病理表现为肌纤维局灶溶解坏死，未见明显炎细胞浸润；基因检测未发现致病基因。经激素及免疫抑制剂治疗后康复。结论：儿童免疫介导的坏死性肌病呈不典型肌炎病理改变，需与遗传性肌病鉴别，激素联合免疫抑制剂及IVIG是治疗的有效方法。
Objective: To improve our understanding to idiopathic inflammatory myositis (IIM) in children. Methods: The clinical chearateristics, pathologic change and treatment process were analyzed in a pediatric patient with idiopathic inflammatory myositis. Results: A 9-year-old girl. Proteinuria was the first symptom at onset, she had muscle pain and amyasthenia of double limbs, laboratory tests suggested creatine kinase and myoglobin increased significantly. Electromyogram test showed myogenic lesion. Muscle biopsy showed focal dissolution of necrotic fibers, no obvious inflammatory infiltrates. No pathogenicity gene was found. The patient recovered after the combination therapy of corticosteroid and immunosuppression. Conclusions: The differential diagnosis between immunemediated necrotizing myopathy and genetic muscle disease in children is important for the atypical pathologic change in immunemediated necrotizing myopathy. The combination of corticosteroid with immunosuppressive medications and intravenous immunoglobulins is effective.