中国麻风皮肤病杂志 ›› 2024, Vol. 40 ›› Issue (1): 5-8.doi: 10.12144/zgmfskin202401005

• 论著 • 上一篇    下一篇

JAK1/2抑制剂治疗成人皮肌炎的疗效和安全性评价

林俊杰*,戚春燕*,李阳,佘欣妍,蔡丽婷,王晓华,陈永锋   

  1. 南方医科大学皮肤病医院皮肤科,广东广州,510091
    *共同第一作者
  • 出版日期:2024-01-15 发布日期:2023-12-25

Efficacy and safety of JAK1/2 inhibitor in the treatment of adult dermatomyositis

LIN Junjie*, QI Chunyan*, LI Yang, SHE Xinyan, CAI Liting, WANG Xiaohua, CHEN Yongfeng   

  1. Department of Dermatology, Dermatology Hospital, Southern Medical University, Guangzhou 510091, China
    *Co-first author
  • Online:2024-01-15 Published:2023-12-25

摘要: 目的:评价JAK抑制剂治疗成人皮肌炎(DM)患者的疗效和安全性。方法:收集2020年5月至2022年12月,在南方医科大学皮肤病医院皮肤科门诊就诊的DM患者。开始治疗前由皮肤科医生完成DM皮损范围和严重程度指数(CDASI)评分,同时留取患者皮疹照片并行血常规、生化指标、抗核抗体、抗核抗体谱、肌炎特异性抗体检查,除外禁忌后予口服JAK1/2抑制剂2 mg/d治疗12周。治疗开始后于第4、8、12周随访,评估CDASI评分,复查相关指标,并记录出现的不良反应事件。结果:纳入7例DM患者,6例患者完成12周的药物治疗和随访观察。CDASI评分开始治疗后均呈持续下降趋势,持续改善可维持至第12周。治疗12周后,部分有肌痛和/或肌无力的患者症状得到改善。部分患者肌酶指标下降,但无统计学意义。随访期间患者无严重不良反应。结论:JAK1/2抑制剂有望成为激素控制不佳、需使用激素或其他系统药物的成人DM患者治疗的新选择。

关键词: 皮肌炎, Janus激酶类, 蛋白激酶抑制剂, JAK抑制剂

Abstract: Objective: To evaluate the efficacy and safety of JAK1/2 inhibitors in the treatment of adult patients with dermatomyositis (DM). Methods: Patients with DM who visited the Department of Dermatology Dermatology Hospital of Southern Medical University Hospital of Dermatology from May 2020 to December 2022 were collected. The Disease Area and Severity Index (CDASI) score was assessed by dermatologist before starting treatment. Meanwhile, skin rash photos were collected and blood routine, biochemical indexes, anti-nuclear antibody, anti-nuclear antibody spectrum, and myositis specific antibody were examined. After contraindications were excluded, oral baritinib 2 mg/d was administered for 12 weeks.  At 4, 8, and 12 weeks after treatment, CDASI scores were evaluated, relevant indicators were reviewed, and adverse events were recorded. Results: Seven patients with DM were included, and six patients completed 12 weeks of drug treatment and follow-up observation. The CDASI scores of all the patients showed a continuous downward trend after treatment, and the continuous improvement could be maintained until the 12th week. Some patients showed a decrease in muscle enzyme index, but it was not statistically significant. After 12 weeks of treatment, some patients with myalgia and/or muscle weakness improved their symptoms. The index of muscle enzyme decreased in some patients, but there was no statistical significance. There were no serious adverse events in patients during the follow-up period. Conclusion: JAK1/2 inhibitors may provide a safer and more effective option for the adult DM patients with poor response to hormone or need to be treated with hormones or other systemic drugs.

Key words: dermatomyositis, Janus kinases, protein kinase inhibitors, JAK1/2 inhibitors