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Review
. 2017 Feb;76(2):329-340.
doi: 10.1136/annrheumdis-2016-209247. Epub 2016 Aug 11.

Consensus-based recommendations for the management of juvenile dermatomyositis

Felicitas Bellutti Enders  1   2 , Brigitte Bader-Meunier  3 , Eileen Baildam  4 , Tamas Constantin  5 , Pavla Dolezalova  6 , Brian M Feldman  7 , Pekka Lahdenne  8 , Bo Magnusson  9 , Kiran Nistala  10 , Seza Ozen  11 , Clarissa Pilkington  10 , Angelo Ravelli  12 , Ricardo Russo  13 , Yosef Uziel  14 , Marco van Brussel  15 , Janjaap van der Net  15 , Sebastiaan Vastert  1 , Lucy R Wedderburn  10 , Nicolaas Wulffraat  1 , Liza J McCann  4 , Annet van Royen-Kerkhof  1
Affiliations
Review

Consensus-based recommendations for the management of juvenile dermatomyositis

Felicitas Bellutti Enders et al. Ann Rheum Dis. 2017 Feb.

Abstract

Background: In 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.

Objectives: To provide recommendations for diagnosis and treatment of JDM.

Methods: Recommendations 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.

Results: In 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.

Conclusions: The 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.

Keywords: Autoimmune Diseases; Dermatomyositis; Treatment.

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Conflict of interest statement

FBE—Valeria e Ettore Bossi Foundation. EB—speaker bureau for Roche/ Chugai, Ad Board for Abbvie and Pfizer. BMF—consultant for Novartis, Pfizer, BMS. PL—consultant for BMS, Pfizer. SO—consultant for Novartis, speaker bureau of SOBI. PD—consultant for Roche, speaker bureau for Pfizer, Novartis, grant support from SOBI, Novartis, Abbvie, Roche, Pfizer, Medac. AR—grant/research support from Pfizer and The Myositis Association; consultant for Novartis, Roche; speaker bureau of Abbvie, Novartis, Pfizer, Roche. YU—consultant for Novartis, speaker bureau of Abbvie, Neopharm, Novartis, Roche. NW—grant/research support from EAHC, Abbvie, GSK, Roche, consultant for Genzyme, Novartis, Pfizer, Roche

Figures

Figure 1A
Figure 1A
Flow chart for the treatment of mild/moderate disease in newly diagnosed and refractory patients with juvenile dermatomyositis (JDM).
Figure 1B
Figure 1B
Flow chart for the treatment of severe disease in newly diagnosed and refractory patients with juvenile dermatomyositis (JDM).
See this image and copyright information in PMC

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