Tosun Ayşe, Serdaroğlu Gül, Aslan Mehmet Tayyip, Polat Muzaffer, Akalin Taner, Tekgul Hasan, Gökben Sarenur
Division of Pediatric Neurology, Department of Pediatrics, Ege University Medical School, Bornova, Izmir 35100, Turkey.
Rheumatol Int. 2006 Sep;26(11):1040-3. doi: 10.1007/s00296-006-0141-4. Epub 2006 May 24.
Here we report two patients with severe juvenile dermatomyositis (JDM) complicated with extra musculocutaneous involvement. The first case (a 10-year-old boy) had unusual initial presentation of JDM complicated with interstitial lung disease documented with high-resolution computed tomography. He had a rapidly progressive course and died in 7 weeks after the onset of the disease despite steroid and immunosuppressive treatment. The second case (a 14-year-old boy) was presented with myositis complicated with hepatitis. He also had a chronic course of JDM with unfavorable outcome. It appears that the prognosis of patients with severe JDM is related with the degree of autoimmune vasculitis on extra musculocutaneous involvement.
在此,我们报告两例患有严重青少年皮肌炎(JDM)并伴有肌肉皮肤外受累的患者。第一例(一名10岁男孩)JDM的初始表现不同寻常,高分辨率计算机断层扫描显示其合并间质性肺病。他病情进展迅速,尽管接受了类固醇和免疫抑制治疗,但在疾病发作后7周死亡。第二例(一名14岁男孩)表现为肌炎合并肝炎。他的JDM病程也呈慢性,预后不佳。看来,严重JDM患者的预后与肌肉皮肤外受累的自身免疫性血管炎程度有关。