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儿童关节炎与风湿病研究联盟关于以皮肤为主型疾病的青少年皮肌炎的共识临床治疗方案

Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease.

作者信息

Kim Susan, Kahn Philip, Robinson Angela B, Lang Bianca, Shulman Andrew, Oberle Edward J, Schikler Kenneth, Curran Megan Lea, Barillas-Arias Lilliana, Spencer Charles H, Rider Lisa G, Huber Adam M

机构信息

Division of Pediatric Rheumatology, Benioff Children's Hospital, University of California at San Francisco, 550 16th St, San Francisco, CA, USA.

Division of Pediatric Rheumatology, New York University Langone Medical Center, 550 First Avenue, New York, NY, USA.

出版信息

Pediatr Rheumatol Online J. 2017 Jan 11;15(1):1. doi: 10.1186/s12969-016-0134-0.

Abstract

BACKGROUND

Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript.

METHODS

The Children's Arthritis and Rheumatology Research Alliance (CARRA) is a North American consortium of pediatric rheumatology health care providers. CARRA members collaborated to determine consensus on typical treatments for JDM patients with skin findings without significant weakness, to develop CTPs for this subgroup of patients. We used a combination of Delphi surveys and nominal group consensus meetings to develop these CTPs.

RESULTS

Consensus was reached on patient characteristics and outcome assessment, and CTPs were developed and finalized for patients with skin predominant JDM. Treatment option A included hydroxychloroquine alone, Treatment option B included hydroxychloroquine and methotrexate, and Treatment option C included hydroxychloroquine, methotrexate and corticosteroids.

CONCLUSIONS

Three CTPs were developed for use in children with skin predominant JDM, which reflect typical treatment approaches. These are not considered to be specific recommendations or standard of care. Using the CARRA network and prospective data collection, we will be able to apply statistical methods in the future to allow comparisons of JDM patients following these consensus treatment plans.

摘要

背景

幼年皮肌炎(JDM)是儿童特发性炎性肌病最常见的形式。一部分儿童有JDM皮疹但无明显肌无力,而针对这些儿童的最佳治疗方法尚不清楚。本研究的目的是描述针对有活动性皮疹、无明显肌肉受累的JDM儿童(本文中称为以皮肤表现为主的JDM)制定共识临床治疗方案(CTP)的过程。

方法

儿童关节炎与风湿病研究联盟(CARRA)是北美儿科风湿病医疗服务提供者的一个联盟。CARRA成员共同协作,就有皮肤表现但无明显肌无力的JDM患者的典型治疗方法达成共识,为该亚组患者制定CTP。我们采用德尔菲调查和名义群体共识会议相结合的方式来制定这些CTP。

结果

就患者特征和结局评估达成了共识,并为以皮肤表现为主的JDM患者制定并最终确定了CTP。治疗方案A包括单用羟氯喹,治疗方案B包括羟氯喹和甲氨蝶呤,治疗方案C包括羟氯喹、甲氨蝶呤和皮质类固醇。

结论

为以皮肤表现为主的JDM儿童制定了三种CTP,反映了典型的治疗方法。这些不被视为具体建议或护理标准。利用CARRA网络和前瞻性数据收集,我们未来将能够应用统计方法来比较遵循这些共识治疗方案的JDM患者。

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