Abstract
BackgroundJuvenile Dermatomyositis (JDM) is a rare pediatric autoimmune disease with broad variations of the individual course. Data on the optimal management are mostly lacking. Currently treatment decisions are often based on experts’ opinions. In order to develop consensus-based treatment strategies for JDM in Germany a survey was pursued to analyze the current clinical practice.MethodsAn online survey addressing all members of the Society for Pediatric Rheumatology (GKJR) in Germany and Austria and pediatric neurologists with expertise in JDM was performed in February/March of 2016. The questionnaire consisted of 5 case scenarios including diagnostic criteria, treatment of moderate, severe and refractory JDM, using either multiple choice or a 5-point Likert scale. Basic descriptive statistics were used to analyze the findings.ResultsThe survey was completed by 60 pediatric rheumatologists and 7 pediatric neurologists experienced in the management of JDM. Typical findings allowing a diagnosis were considered to be: typical skin changes, proximal muscle weakness, MRI findings, elevated muscle enzymes, nailfold capillary changes, presence of calcinosis and muscle biopsy. Regarding induction treatment of moderate/severe JDM: 59%/74% opted for intermittent intravenous methylprednisolone (IVMP) pulse therapy, and 21%/40% for conventional high-dose oral glucocorticoids. Methotrexate (MTX) was the preferred disease-modifying conventional anti-rheumatic drug (cDMARD) for moderate and severe JDM. Regarding the management of refractory moderate or severe JDM, intravenous immune globulins, mycophenolate mofetil and rituximab were preferred treatment options.ConclusionThere is consensus about the diagnosis of JDM strongly supported by classic clinical and MRI findings. There is great variety in the treatment of JDM in Germany regarding both induction and maintenance therapy. The development of consensus-based treatment strategies for JDM based on harmonization of current clinical practice is essential in order to allow comparative effectiveness research in the future.
Highlights
Juvenile Dermatomyositis (JDM) is a rare pediatric autoimmune disease with broad variations of the individual course
The authors C.H., F.S. and P.O. opted to develop a survey consisting of 5 case scenarios (23 questions) with the option to extend the survey for an additional case scenario addressing the issue of dystrophic calcification (9 questions)
The survey addressed (1) initial diagnostic measures taken in a patient with probable JDM, making the diagnosis of moderate JDM and initial treatment steps (10 questions), (2) maintenance treatment in patients with moderate JDM (6 questions), (3) treatment of refractory moderate JDM (3 questions), (4) treatment of glucocorticoid-dependent moderate JDM (1 question), (5) initial treatment of severe JDM and treatment of refractory severe JDM (3 questions), and, optionally, (6) management of dystrophic calcification (9 questions) (Additional file 1)
Summary
Juvenile Dermatomyositis (JDM) is a rare pediatric autoimmune disease with broad variations of the individual course. Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy of childhood [1]. Even though it is a rare condition, it is still a major cause of morbidity and mortality among patients with pediatric rheumatic diseases [2, 3]. A European initiative resulted in international consensus-based recommendations and treatment protocols [7, 8]. It is unclear if practice patterns in Germany vary from those in North America or other countries, so that additional data are desirable
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