NEJM:青少年皮肌炎钙化病-病例报道

2019-10-17 xing.T MedSci原创

考虑到患者顽固性青少年皮肌炎和严重的钙化病,开始使用英夫利昔单抗、沙利度胺和氢氧化铝治疗。治疗后两个月,患者的肌肉力量和皮疹褪色有所改善。

患者为一名4岁的青少年皮肌炎女孩,因皮疹和四肢无力被送至风湿科就诊。尽管在另一家医院接受了3年以上的多种药物治疗,包括糖皮质激素、静脉内免疫球蛋白、甲氨蝶呤和环孢霉素的治疗,她仍具有连续的疾病活动且无缓解。

体格检查时,患者的手指、膝盖和肘关节上的皮肤出现红斑改变(发现与Gottron体征相符),眼睛周围有日光性皮疹,手臂和腿部近端肌肉无力,以及可见的弥漫性皮下结节。骨盆的X光片显示广泛的钙化(如图A所示)。大腿的磁共振成像显示肌肉炎症和钙化。实验室检查结果表明,强抗核基质蛋白2(anti-NXP-2)自身抗体阳性,这一发现与青少年皮肌炎的钙化相关。



考虑到患者顽固性青少年皮肌炎和严重的钙化病,开始使用英夫利昔单抗、沙利度胺和氢氧化铝治疗。治疗后两个月,患者的肌肉力量和皮疹褪色有所改善。

在治疗的9个月时,重复的X射线照片显示钙化作用已减弱(如图B所示)。 
 
原始出处:

Jianguo Li,et al.Calcinosis in Juvenile Dermatomyositis.N Engl J Med 2019;https://www.nejm.org/doi/full/10.1056/NEJMicm1809669

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    2020-01-27 lg.zhao
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    2019-10-17 旺医

    顶刊就是顶刊,谢谢梅斯带来这么高水平的研究报道,我们科里同事经常看梅斯,分享梅斯上的信息

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