Dressler F, Frosch M, Mönkemöller K, Thon A, Weissbarth-Riedel E, Horneff G
Kinderklinik, Medizinische Hochschule Hannover, Carl-Neuberg-Straße 1, Hannover,Germany.
Klin Padiatr. 2011 Sep;223(5):280-2. doi: 10.1055/s-0031-1273723. Epub 2011 Apr 20.
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. Diagnostic as 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.
在一项全国性登记项目中,德国在两年时间里收集到38例青少年皮肌炎的新发病例。记录了这些患者的诊断方法以及初始治疗情况。其中25例患者有详细信息。诊断和治疗决策差异很大。25例患者中几乎全部都使用了类固醇,采用口服或静脉冲击疗法,52%的患者还使用了其他免疫抑制药物。我们计划就青少年皮肌炎的诊断和治疗标准制定全国性共识建议。由于青少年皮肌炎罕见,临床试验将不得不基于国际层面开展。