Lang B, Dooley J
Department of Pediatrics, Izaak Walton Killam Children's Hospital, Dalhousie University, Halifax, Nova Scotia, Canada.
J Pediatr. 1996 Mar;128(3):429-32. doi: 10.1016/s0022-3476(96)70295-0.
To review the experience with high-dose intravenous pulse methylprednisolone (IVMP) therapy in patients with juvenile dermatomyositis (JDM) in our institution.
We reviewed the charts of seven consecutive patients (four female subjects; three male subjects; age 3 to 18 years (mean age 8 years) treated for JDM between 1989 and 1992.
All patients met the criteria of Bohan and Peter for JDM and were treated within 3 months of onset of weakness. All received initial treatment with IVMP, 30 mg/kg per day for 3 days, administered weekly for between 1 and 4 weeks. Treatment with orally administered prednisone was required because of deteriorating muscle strength and persistent elevation of creatine kinase activity despite IVMP in five patients, increased skin vasculitis in one patient, and elevated creatine kinase activity with no improvement in strength in one patient.
We conclude that muscle strength in patients with JDM may deteriorate during pulse IVMP therapy when this is used alone as initial treatment, even early in the course of the illness. We recommend either conventional high doses of orally administered corticosteroids or IVMP with oral corticosteroid therapy as initial treatment of JDM.
回顾我院青少年皮肌炎（JDM）患者大剂量静脉注射脉冲式甲泼尼龙（IVMP）治疗的经验。
我们回顾了1989年至1992年间连续治疗的7例JDM患者（4例女性，3例男性；年龄3至18岁，平均年龄8岁）的病历。
所有患者均符合博汉和彼得关于JDM的标准，且在肌无力发作3个月内接受治疗。所有患者均接受IVMP初始治疗，每日30mg/kg，共3天，每周给药1至4周。5例患者尽管接受了IVMP治疗，但由于肌力恶化和肌酸激酶活性持续升高，1例患者皮肤血管炎加重，1例患者肌酸激酶活性升高但肌力无改善，因此需要口服泼尼松治疗。
我们得出结论，当单独使用脉冲IVMP治疗作为初始治疗时，即使在疾病早期，JDM患者的肌力在治疗期间也可能恶化。我们建议使用常规高剂量口服糖皮质激素或IVMP联合口服糖皮质激素治疗作为JDM的初始治疗方法。
