难治性皮肤性皮肌炎的治疗：托法替尼的 Janus 激酶抑制作用的潜在作用。
Management of refractory cutaneous dermatomyositis: potential role of Janus kinase inhibition with tofacitinib.
机构信息
Myositis Center and Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine.
Department of Rheumatology, VA Pittsburgh Healthcare System, Pittsburgh, PA, USA.
出版信息
Rheumatology (Oxford). 2019 Jun 1;58(6):1011-1015. doi: 10.1093/rheumatology/key366.
OBJECTIVES
Some patients with cutaneous DM demonstrate incomplete responses to conventional therapy while some, including those with extra-cutaneous manifestations, experience disease recurrences. Janus kinase/signal transducers and activators of transcription pathway inhibition has been reported to mitigate IFN signalling, which is thought to contribute to disease pathogenesis in DM. Four cases of refractory DM responsive to tofacitinib have been reported in the literature. Our case series investigated the use of tofacitinib in refractory cutaneous DM.
METHODS
Our case series includes four subjects with refractory DM who received tofaticinib after failure of several immunosuppressive and immunomodulatory agents.
RESULTS
All four subjects responded well to tofacitinib with significant improvement in cutaneous and extra-cutaneous manifestations.
CONCLUSION
Tofacitinib can improve cutaneous and inflammatory articular manifestations in refractory DM.
目的
部分皮肤型 DM 患者对常规治疗反应不完全，而部分患者（包括有皮肤外表现者）则出现疾病复发。Janus 激酶/信号转导和转录激活因子通路抑制已被报道可减轻 IFN 信号，这被认为有助于 DM 的发病机制。文献中报道了 4 例对托法替尼有反应的难治性 DM 病例。我们的病例系列研究了托法替尼在难治性皮肤型 DM 中的应用。
方法
我们的病例系列包括 4 例难治性 DM 患者，他们在几种免疫抑制和免疫调节药物治疗失败后接受了托法替尼治疗。
结果
所有 4 例患者对托法替尼反应良好，皮肤和皮肤外表现均有显著改善。
结论
托法替尼可改善难治性 DM 的皮肤和炎症性关节表现。
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