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

Abstract

BackgroundJuvenile 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.MethodsThe 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.ResultsConsensus 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.ConclusionsThree 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.

Treatment of Juvenile Dermatomyositis: An Update

“…As there are few data from randomised controlled trials to guide management decisions in the children with IIM, recently several algorithms for the treatment of moderately severe JDM were developed using international expert opinion [38][39][40]. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) has described several treatment strategies for initial therapy for JDM based upon a North American survey of practice [39], while recently consensus-based recommendations for the management of JDM were published by a…”

Juvenile dermatomyositis: Latest advances

“…This is particularly important in rare diseases where evidence is limited and randomised controlled trials are not possible due to small patient numbers. CARRA have recently extended CTPs in JDM to include treatment of persistent skin rash and treatment of skin predominant disease (18,19). Table 4 summarises the available evidence for drugs commonly used in the treatment of JDM, highlighting the sparsity of evidence on which clinical practice is based.…”

Submaximal Exercise Capacity in Juvenile Dermatomyositis after Longterm Disease: The Contribution of Muscle, Lung, and Heart Involvement

“…• High doses of Prednisone/Methylprednisolone (54,59) • Methotrexate (53) Other commonly used drugs involve cyclosporine, intravenous immunoglobulin (IVIG), and hydroxychloroquine, depending on the presentation of clinical manifestations (51,53,55).…”