Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Foundation Trust.
Infection, Immunity and Inflammation, UCL Great Ormond Street Institute of Child Health.
Curr Opin Rheumatol. 2018 Nov;30(6):650-654. doi: 10.1097/BOR.0000000000000538.
The aim of this article is to provide a summary of the recent therapeutic advances and the latest research on outcome measures for juvenile dermatomyositis (JDM).
Several new international studies have developed consensus-based guidelines on diagnosis, outcome measures and treatment of JDM to standardize and improve patient care. Myositis-specific antibodies together with muscle biopsy histopathology may help the clinician to predict disease outcome. A newly developed MRI-based scoring system has been developed to standardize the use of MRI in assessing disease activity in JDM. New data regarding the efficacy and safety of rituximab, especially for skin disease, and cyclophosphamide in JDM support the use of these medications for severe refractory cases.
International network studies, new biomarkers and outcome measures have led to significant progress in understanding and managing the rare inflammatory myositis conditions such as JDM.
本文旨在总结青少年皮肌炎(JDM)治疗进展和最新结局评估指标研究进展。
数项新的国际研究针对 JDM 的诊断、结局评估指标和治疗制定了基于共识的指南,以规范和改善患者管理。肌炎特异性抗体结合肌肉活检组织病理学检查,可能有助于临床医生预测疾病结局。新开发的基于 MRI 的评分系统有助于规范 MRI 在 JDM 疾病活动评估中的应用。有关利妥昔单抗(特别是治疗皮肤疾病)和环磷酰胺治疗 JDM 的疗效和安全性的新数据支持将这些药物用于严重难治性病例。
国际网络研究、新的生物标志物和结局评估指标使得人们对包括 JDM 在内的罕见炎性肌病的认识和管理取得了显著进展。