Senju Ayako, Shimono Masayuki, Shiota Naoki, Ishii Masahiro, Takano Kenichi, Takano Shiho, Takei Shuji, Nerome Yasuhito
Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Fukuoka.
No To Hattatsu. 2011 Jul;43(4):309-12.
We herein report a 3 year-old boy, who showed proximal muscle weakness and pain at the age of one and a-half years. When he visited our hospital at the age of 1 year and 11 months, he could hardly move by himself. He also had difficulty in swallowing and suffered from multiple dermal ulcers. His blood test showed slightly elevated muscle enzyme activity, and magnetic resonance imaging suggested severe inflammation of the muscles. Radiological examination proved hypoperistalsis of the esophagus. With additional skin and muscle biopsies, we diagnosed him with juvenile dermatomyositis (JDM). Methyl-prednisolone pulse therapy was not effective enough, thus oral methotrexate, cyclosporine A and monthly cyclophosphamide pulse therapy were added. After the fourth cyclophosphamide pulse therapy, his muscular strength was restored, and the ulcers healed dramatically. Due to scarcity of severe cases, neither standardized classification nor grading system for severity in JDM has ever been established, which perplexes physicians in finding the best therapeutic strategy. Further investigation, experience and efforts are necessary to standardize an evaluating system and therapeutic strategy against JDM.
我们在此报告一名3岁男孩，他在一岁半时出现近端肌无力和疼痛。当他1岁11个月就诊于我院时，几乎无法自主活动。他还存在吞咽困难，并患有多处皮肤溃疡。他的血液检查显示肌肉酶活性略有升高，磁共振成像提示肌肉严重炎症。放射学检查证实食管蠕动减弱。经皮肤和肌肉活检，我们诊断他为幼年皮肌炎（JDM）。甲泼尼龙冲击疗法效果欠佳，因此加用了口服甲氨蝶呤、环孢素A及每月一次的环磷酰胺冲击疗法。在第四次环磷酰胺冲击疗法后，他的肌力恢复，溃疡显著愈合。由于重症病例稀缺，JDM从未建立过标准化的分类或严重程度分级系统，这给医生寻找最佳治疗策略带来了困扰。有必要进行进一步的研究、积累经验并付出努力，以规范JDM的评估系统和治疗策略。
