Artikel
Variability in the Diagnosis and Treatment of Juvenile Dermatomyositis in Germany – a Survey among Pediatric Rheumatologists and Child Neurologists
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Autoren
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Claas Hinze
- Universitätsklinikum Münster, Klinik für Pädiatrische Rheumatologie und Immunologie, Münster
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Prasad Thomas Oommen
- Universitätsklinikum Düsseldorf, Zentrum für Kinder- und Jugendmedizin, Klinik für Kinder-Onkologie, Hämatologie und klinische Immunologie, Düsseldorf
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Fabian Speth
- Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen
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Johannes Peter Haas
- Deutsches Zentrum für Kinder- und Jugendrheumatologie Zentrum für Schmerztherapie junger Menschen, Garmisch-Partenkirchen
| Veröffentlicht: | 29. August 2016 |
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Gliederung
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Background: Juvenile Dermatomyositis (JDM) is an extremely rare pediatric rheumatic disease and data on its optimal management are mostly lacking. In order to develop consensus treatment protocols (CTPs) for JDM in Germany an understanding of the current clinical practice is essential.
Methods: We designed an online survey addressing all members of the Society for Pediatric Rheumatology (GKJR) in Germany and child neurologists with experience in the treatment of JDM in February/March of 2016. The questionnaire consisted of 6 case scenarios addressing diagnosis, treatment of moderate, severe and refractory JDM, and calcinosis, using either multiple choice or a 5-point Likert scale. Descriptive statistics was used to analyze the findings.
Results: The survey was completed by 59 members of the GKJR and 7 child neurologists. The most important characteristics for the diagnosis of JDM in clinical practice based on a 5-point Likert scale (1=essential, 5=not important at all) were considered to be: typical skin findings (1.30 weighted average), proximal muscle weakness (1.51), typical MRI findings (1.61) and elevated muscle enzymes (1.67). Induction treatment of moderate (severe) JDM was highly variable: 28.4% (41.9%) use at least a single 3-day course of IV methylprednisolone (IVMP) pulse therapy, 68.7% (72.6%) use intermittent IVMP pulse therapy. High-dose, medium-dose and low-dose oral steroids were used by 25.4% (38.7%), 29.9% (30.7%) and 26.9% (4.8%), respectively. The following steroid-sparing drugs were used: Methotrexate 76.6% (91.3%), hydroxychloroquine 20.9% (54.8%), azathioprine 9.0% (11.3%), mycophenolate mofetil (MMF) 4.5% (8.1%), cyclosporine 1.5% (6.5%), intravenous immunoglobulins (IVIG) 14.9% (61.3%), rituximab 0% (9.7%) and/or cyclophosphamide 0% (4.8%). Regarding JDM refractory to steroids and methotrexate, the following treatment preferences exist based on a 5-point Likert scale: IVIG (1.47 weighted average), MMF (1.86), rituximab (2.00), cyclosporine (2.28) and intensified steroid therapy (2.35).
Conclusion: There is consensus that the diagnosis of JDM, in addition to classic clinical findings, is strongly supported by typical MRI findings. Treatment of JDM in Germany is highly variable both in the induction and the maintenance treatment phases. IVIG is a commonly employed treatment in refractory JDM. The development of CTPs for the treatment of JDM is desirable in order to harmonize treatment and allow comparative effectiveness research.
