Riley P, McCann L J, Maillard S M, Woo P, Murray K J, Pilkington C A
Juvenile Dermatomyositis Research Centre, Institute of Child Health UCL, 30 Guilford Street, London WC1N 1EH, UK.
Rheumatology (Oxford). 2008 Jun;47(6):877-80. doi: 10.1093/rheumatology/ken074. Epub 2008 Apr 9.
Some juvenile dermatomyositis (JDM) patients have a disease course which is refractory to multiple drug treatments. Prolonged disease activity is associated with increased mortality and morbidity. TNF-alpha has been identified in high levels in JDM patients who have a long disease course and calcinosis. We assessed the response of five refractory JDM patients to the anti-TNF-alpha monoclonal antibody, infliximab.
For all five patients intravenous infliximab was initially given at a dose of 3 mg/kg. Further doses were then given at weeks 2, 6 and every 8 weeks thereafter. The dose and frequency were tailored in accordance with clinical response. Clinical and laboratory data were collected prospectively.
We report results between 8 and 30 months after starting infliximab. Improvements were seen in all five patients as shown by positive changes in physician visual analogue scale (VAS), Childhood Myositis Assessment Score (CMAS), Childhood Health Assessment Questionnaire (CHAQ), joint range of movement and, in some, regression of calcinosis and skin signs. There were no major side effects observed with addition of infliximab to the therapeutic regime.
Major clinical benefit was demonstrated after the initiation of infliximab in all five cases of refractory JDM.
一些青少年皮肌炎（JDM）患者的病程对多种药物治疗均难治。疾病活动期延长与死亡率和发病率增加相关。在病程长且有钙质沉着的JDM患者中已发现肿瘤坏死因子-α（TNF-α）水平升高。我们评估了5例难治性JDM患者对抗TNF-α单克隆抗体英夫利昔单抗的反应。
对所有5例患者，英夫利昔单抗初始静脉给药剂量为3 mg/kg。随后在第2周、第6周及此后每8周给药一次。剂量和频率根据临床反应进行调整。前瞻性收集临床和实验室数据。
我们报告了开始使用英夫利昔单抗后8至30个月的结果。所有5例患者均有改善，表现为医生视觉模拟量表（VAS）、儿童肌炎评估评分（CMAS）、儿童健康评估问卷（CHAQ）、关节活动范围出现积极变化，部分患者钙质沉着和皮肤体征消退。在治疗方案中加用英夫利昔单抗未观察到重大副作用。
在所有5例难治性JDM患者中，开始使用英夫利昔单抗后均显示出主要临床获益。
