PDF(Pubmed)
Abstract：
OBJECTIVE: To evaluate the effectiveness and safety of upadacitinib in treatment-refractory inflammatory myositis.
METHODS: Patients with refractory inflammatory myositis treated with upadacitinib from a single urban centre in Vancouver, British Columbia, Canada, were included from September 2020 to June 2023. The medical records of these patients were retrospectively reviewed.
RESULTS: 10 total patients were identified for review, including 5 classic dermatomyositis (DM), 3 amyopathic DM (ADM) and 2 antisynthetase syndrome. The patients failed an average of four immunosuppressants before initiation of upadacitinib. Three had prior Janus kinase inhibitor therapy with tofacitinib. In the classic DM and ADM aggregate group, upadacitinib offered clinically and statistically significant cutaneous improvement. Lack of active muscle disease at baseline precluded analysis of the effect of upadacitinib on muscle weakness. Nine patients remained on upadacitinib at the end of the study period. One patient discontinued upadacitinib due to severe facial acne.
CONCLUSIONS: Upadacitinib appears to be effective in targeting cutaneous manifestations of refractory inflammatory DM. Further research is still needed to validate its efficacy on a broader population scale.
METHODS: Patients with refractory inflammatory myositis treated with upadacitinib from a single urban centre in Vancouver, British Columbia, Canada, were included from September 2020 to June 2023. The medical records of these patients were retrospectively reviewed.
RESULTS: 10 total patients were identified for review, including 5 classic dermatomyositis (DM), 3 amyopathic DM (ADM) and 2 antisynthetase syndrome. The patients failed an average of four immunosuppressants before initiation of upadacitinib. Three had prior Janus kinase inhibitor therapy with tofacitinib. In the classic DM and ADM aggregate group, upadacitinib offered clinically and statistically significant cutaneous improvement. Lack of active muscle disease at baseline precluded analysis of the effect of upadacitinib on muscle weakness. Nine patients remained on upadacitinib at the end of the study period. One patient discontinued upadacitinib due to severe facial acne.
CONCLUSIONS: Upadacitinib appears to be effective in targeting cutaneous manifestations of refractory inflammatory DM. Further research is still needed to validate its efficacy on a broader population scale.
摘要：
目的：评价upadacitinib治疗难治性炎性肌炎的有效性和安全性。
方法：在温哥华的一个城市中心接受upadacitinib治疗的难治性炎性肌炎患者，不列颠哥伦比亚省,加拿大,包括2020年9月至2023年6月。对这些患者的病历进行回顾性分析。
结果：共确定10名患者进行审查，包括5个典型的皮肌炎（DM），3肌病性DM（ADM）和2抗合成酶综合征。在开始upadacitinib之前，患者平均4种免疫抑制剂失败。三个人之前曾接受过托法替尼的Janus激酶抑制剂治疗。在经典的DM和ADM聚合组中，upadacitinib提供了临床和统计学上显着的皮肤改善。基线时缺乏活动性肌肉疾病，无法分析upadacitinib对肌肉无力的影响。在研究期结束时，9名患者仍在服用upadacitinib。一名患者因严重面部痤疮而停用upadacitinib。
结论：Upadacitinib似乎可有效靶向难治性炎性DM的皮肤表现。仍需要进一步的研究来验证其在更广泛人群范围内的功效。
方法：在温哥华的一个城市中心接受upadacitinib治疗的难治性炎性肌炎患者，不列颠哥伦比亚省,加拿大,包括2020年9月至2023年6月。对这些患者的病历进行回顾性分析。
结果：共确定10名患者进行审查，包括5个典型的皮肌炎（DM），3肌病性DM（ADM）和2抗合成酶综合征。在开始upadacitinib之前，患者平均4种免疫抑制剂失败。三个人之前曾接受过托法替尼的Janus激酶抑制剂治疗。在经典的DM和ADM聚合组中，upadacitinib提供了临床和统计学上显着的皮肤改善。基线时缺乏活动性肌肉疾病，无法分析upadacitinib对肌肉无力的影响。在研究期结束时，9名患者仍在服用upadacitinib。一名患者因严重面部痤疮而停用upadacitinib。
结论：Upadacitinib似乎可有效靶向难治性炎性DM的皮肤表现。仍需要进一步的研究来验证其在更广泛人群范围内的功效。
