Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists
Abstract Background Juvenile 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 treatme...
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| Format: | Article |
| Language: | English |
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BMC
2018-06-01
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| Series: | Pediatric Rheumatology Online Journal |
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| Online Access: | http://link.springer.com/article/10.1186/s12969-018-0256-7 |
| _version_ | 1811192508696231936 |
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| author | Claas H. Hinze Fabian Speth Prasad T. Oommen Johannes-Peter Haas |
| author_facet | Claas H. Hinze Fabian Speth Prasad T. Oommen Johannes-Peter Haas |
| author_sort | Claas H. Hinze |
| collection | DOAJ |
| description | Abstract Background Juvenile 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. Methods An 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. Results The 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. Conclusion There 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. |
| first_indexed | 2024-04-11T23:54:47Z |
| format | Article |
| id | doaj.art-0bbb06954dde409ca2b90111ff55414b |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-04-11T23:54:47Z |
| publishDate | 2018-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-0bbb06954dde409ca2b90111ff55414b2022-12-22T03:56:24ZengBMCPediatric Rheumatology Online Journal1546-00962018-06-011611810.1186/s12969-018-0256-7Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologistsClaas H. Hinze0Fabian Speth1Prasad T. Oommen2Johannes-Peter Haas3Department of Pediatric Rheumatology and Immunology, University Hospital MünsterDivision of Pediatric Rheumatology, University MedicineCenter of Child and Adolescent Health, Department of Pediatric Oncology, Hematology and Clinical Immunology, Heinrich-Heine University DuesseldorfGerman Center for Pediatric and Adolescent RheumatologyAbstract Background Juvenile 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. Methods An 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. Results The 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. Conclusion There 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.http://link.springer.com/article/10.1186/s12969-018-0256-7DermatomyositisSurveys and questionnairesPractice patternsPhysiciansGlucocorticoidsMethotrexate |
| spellingShingle | Claas H. Hinze Fabian Speth Prasad T. Oommen Johannes-Peter Haas Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists Pediatric Rheumatology Online Journal Dermatomyositis Surveys and questionnaires Practice patterns Physicians Glucocorticoids Methotrexate |
| title | Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists |
| title_full | Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists |
| title_fullStr | Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists |
| title_full_unstemmed | Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists |
| title_short | Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists |
| title_sort | current management of juvenile dermatomyositis in germany and austria an online survey of pediatric rheumatologists and pediatric neurologists |
| topic | Dermatomyositis Surveys and questionnaires Practice patterns Physicians Glucocorticoids Methotrexate |
| url | http://link.springer.com/article/10.1186/s12969-018-0256-7 |
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