Consensus-based recommendations for the management of juvenile dermatomyositis

Abstract

BackgroundIn 2012, a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile dermatomyositis (JDM) is a rare disease within the group of paediatric rheumatic diseases (PRDs) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment regimens differ throughout Europe.ObjectivesTo provide recommendations for diagnosis and treatment of JDM.MethodsRecommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was constituted, consisting of 19 experienced paediatric rheumatologists and 2 experts in paediatric exercise physiology and physical therapy, mainly from Europe. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using nominal group technique. Recommendations were accepted if >80% agreement was reached.ResultsIn total, 7 overarching principles, 33 recommendations on diagnosis and 19 recommendations on therapy were accepted with >80% agreement among experts. Topics covered include assessment of skin, muscle and major organ involvement and suggested treatment pathways.ConclusionsThe SHARE initiative aims to identify best practices for treatment of patients suffering from PRD. Within this remit, recommendations for the diagnosis and treatment of JDM have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JDM throughout Europe.

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…”

“…JDM rash is known to be a photosensitive rash and sun exposure is known to exacerbate disease flares. It is thus recommended the daily use of high factor sun sunscreen [40]. Concomitant use of calcium and vitamin D supplements is recommended to prevent osteoporosis, as long term high doses of corticosteroids are usually required for the treatment of IIMs [124,125].…”

“…use of MTX in these regions [58]. In general, for children with JDM, MTX therapy is started at a dose of 15-20 mg/m 2 /week, and given by the subcutaneous route [40]. Around 70% of patients with JDM benefit significantly from MTX therapy, with the maximum therapeutic effect usually becoming apparent 3 months after the beginning of treatment [57].…”

The Vasculopathy of Juvenile Dermatomyositis

“…It is important to highlight that these CTPs do not consist of therapeutic guidelines per se , but they represent arms for proposed comparative effectiveness studies, developed via consensus methodologies. Moreover, evidence-driven consensus-based recommendations for diagnosis and treatment of JDM have also been published as part of a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) ( 67 ).…”

“…More adverse effects were seen with ciclosporin than MTX, supporting the usual practice of corticosteroid and MTX as first line treatment ( 73 ). Intravenous immunoglobulin (IVIG) is recommended for patients with JDM refractory to steroids and methotrexate ( 67 , 68 ). An alternative DMARD suggested for severe/refractory cases of JDM or where methotrexate is not tolerated, is mycophenolate mofetil ( 67 ), beneficial for both muscle and skin disease.…”