Hinze Claas H, Oommen Prasad T, Dressler Frank, Urban Andreas, Weller-Heinemann Frank, Speth Fabian, Lainka Elke, Brunner Jürgen, Fesq Heike, Foell Dirk, Müller-Felber Wolfgang, Neudorf Ulrich, Rietschel Christoph, Schwarz Tobias, Schara Ulrike, Haas Johannes-Peter
Department of Pediatric Rheumatology and Immunology, University Hospital Münster, Albert-Schweitzer-Campus 1, Building D3, 48149, Münster, Germany.
Department of Pediatric Oncology, Hematology and Clinical Immunology, University Hospital Düsseldorf, Düsseldorf, Germany.
Pediatr Rheumatol Online J. 2018 Jun 25;16(1):40. doi: 10.1186/s12969-018-0257-6.
Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and a major cause of morbidity among children with pediatric rheumatic diseases. The management of JDM is very heterogeneous. The JDM working group of the Society for Pediatric Rheumatology (GKJR) aims to define consensus- and practice-based strategies in order to harmonize diagnosis, treatment and monitoring of JDM.
The JDM working group was established in 2015 consisting of 23 pediatric rheumatologists, pediatric neurologists and dermatologists with expertise in the management of JDM. Current practice patterns of management in JDM had previously been identified via an online survey among pediatric rheumatologists and neurologists. Using a consensus process consisting of online surveys and a face-to-face consensus conference statements were defined regarding the diagnosis, treatment and monitoring of JDM. During the conference consensus was achieved via nominal group technique. Voting took place using an electronic audience response system, and at least 80% consensus was required for individual statements.
Overall 10 individual statements were developed, finally reaching a consensus of 92 to 100% regarding (1) establishing a diagnosis, (2) case definitions for the application of the strategies (moderate and severe JDM), (3) initial diagnostic testing, (4) monitoring and documentation, (5) treatment targets within the context of a treat-to-target strategy, (6) supportive therapies, (7) explicit definition of a treat-to-target strategy, (8) various glucocorticoid regimens, including intermittent intravenous methylprednisolone pulse and high-dose oral glucocorticoid therapies with tapering, (9) initial glucocorticoid-sparing therapy and (10) management of refractory disease.
Using a consensus process among JDM experts, statements regarding the management of JDM were defined. These statements and the strategies aid in the management of patients with moderate and severe JDM.
幼年皮肌炎（JDM）是儿童期最常见的炎性肌病，也是小儿风湿性疾病患儿发病的主要原因。JDM的治疗方法差异很大。儿科风湿病学会（GKJR）的JDM工作组旨在确定基于共识和实践的策略，以统一JDM的诊断、治疗和监测。
JDM工作组于2015年成立，由23名在JDM管理方面具有专业知识的儿科风湿病学家、儿科神经科医生和皮肤科医生组成。JDM目前的治疗实践模式此前已通过对儿科风湿病学家和神经科医生的在线调查确定。通过在线调查和面对面的共识会议组成的共识过程，确定了关于JDM诊断、治疗和监测的声明。在会议期间，通过名义群体技术达成了共识。使用电子观众反应系统进行投票，每项声明至少需要80%的共识。
总共制定了10项单独声明，最终在以下方面达成了92%至100%的共识：（1）建立诊断；（2）策略应用的病例定义（中度和重度JDM）；（3）初始诊断测试；（4）监测和记录；（5）在目标治疗策略背景下的治疗目标；（6）支持性治疗；（7）目标治疗策略的明确定义；（8）各种糖皮质激素治疗方案，包括间歇性静脉注射甲泼尼龙脉冲治疗和逐渐减量的高剂量口服糖皮质激素治疗；（9）初始糖皮质激素节约治疗；（10）难治性疾病的管理。
通过JDM专家之间的共识过程，确定了关于JDM管理的声明。这些声明和策略有助于中度和重度JDM患者的管理。
