TY  - JOUR
A1  - Hinze, Claas Heinrich
A1  - Oommen, Prasad Thomas
A1  - Dressler, Frank
A1  - Urban, Andreas
A1  - Weller-Heinemann, Frank
A1  - Speth, Fabian
A1  - Lainka, Elke
A1  - Brunner, Jürgen
A1  - Fesq, Heike
A1  - Föll, Dirk
A1  - Müller-Felber, Wolfgang
A1  - Neudorf, Ulrich
A1  - Rietschel, Christoph
A1  - Schwarz, Tobias
A1  - Schara, Ulrike
A1  - Haas, Johannes-Peter
T1  - Development of practice and consensus-based strategies including a treat-to-target approach for the management of moderate and severe juvenile dermatomyositis in Germany and Austria
T2  - Pediatric rheumatology
N2  - Background: 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.
Methods: 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.
Results: 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.
Conclusion: 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.
KW  - Dermatomyositis
KW  - Child
KW  - Consensus
KW  - Diagnosis
KW  - Antirheumatic agents
KW  - Comparative effectiveness research
Y1  - 2018
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/46806
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-468066
SN  - 1546-0096
N1  - Open Access: This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
VL  - 16
IS  - 1, Art. 40
SP  - 1
EP  - 9
PB  - BioMed Central
CY  - London
ER  -