Results of the German ESPED-Recording of New Patients with Juvenile Dermatomyositis (JDM)

Abstract

In a nation-wide registration project 38 incident cases of juvenile dermatomyositis were collected in Germany over a 2-year-period. Diagnostic methods as well as the primary treatment for these patients were recorded. Detailed information was available for 25 of these patients. Diagnostias well as therapeutic decisions varied widely. Steroids were used in almost all of the 25 patients either as oral or as parenteral pulse therapy, additional immunosuppressive drugs were used in 52%. We plan to establish national consensus recommendations for diagnostic and therapeutic standards in JDM. Due to the rarity of JDM clinical trials will have to be performed on an international basis.

Zusammenfassung

In einer nationalen deutschen ESPED-Erhebung wurden 38 neue Fälle von juveniler Dermatomyositis (JDM) in 2 Jahren gemeldet. Die diagnostischen und initialen therapeutischen Schritte wurden per Fragebögen erfasst. Detaillierte Angaben lagen zu 25 Patienten vor. Die diagnostischen und therapeutischen Maßnahmen unterschieden sich deutlich. Steroide wurden bei fast allen der 25 Patienten oral oder parenteral in Pulsen eingesetzt, eine zusätzliche medikamentöse Immunsuppression erfolgte bei 52%. Wir planen nationale Konsensusleitlinien für diagnostische und therapeutische Standards für die JDM zu erstellen. Aufgrund der Seltenheit der Erkrankung müssen klinische Studien international erfolgen.

juvenile - dermatomyositis - diagnosis - therapy

juvenile - Dermatomyositis - Diagnose - Therapie -

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