Abstract
BackgroundJuvenile 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.MethodsThe 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.ResultsOverall 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.ConclusionUsing 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.
Highlights
Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and a major cause of morbidity among children with pediatric rheumatic diseases
Consensus treatment plans have been established in North America by the Childhood Arthritis & Rheumatology Research Alliance (CARRA) and consensus-based treatment recommendations were developed by the Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) initiative [16, 17]
Expert panel The expert status of the panel members is demonstrated by the fact that centers represented in the panel contributed more than half of all patients with JDM documented within the National Pediatric Rheumatic Disease Database (“Kerndokumentation rheumakranker Kinder und Jugendlicher”)
Summary
Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and a major cause of morbidity among children with pediatric rheumatic diseases. Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy of childhood and a major cause of morbidity and mortality among patients with pediatric rheumatic diseases [1]. Only few formally approved treatment options exist for the treatment of dermatomyositis (DM), in Germany, including various glucocorticoids (methylprednisolone, prednisolone, prednisone, dexamethasone, triamcinolone), azathioprin [AZA], and, under certain conditions, IVIG) [15]. None of these medications have been approved for the use in JDM. The concept of treat-to-target and tight control has been a cornerstone of current treatment recommendations for rheumatoid arthritis, systemic lupus erythematosus and gout, and has been discussed for pediatric rheumatic diseases as well [20,21,22,23,24]
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