Elst Elisabeth F, Kamphuis Sylvia S M, Prakken Berent J, Wulffraat Nicolas M, van der Net Janjaap, Peters A C Boudewyn, Kuis Wietse
Department of Pediatric Immunology, University Medical Center of Utrecht, Wilhelmina Children's Hospital, mailbox KC.03.063.0, 3508 AB Utrecht, The Netherlands.
J Rheumatol. 2003 Sep;30(9):2059-63.
We present 3 patients with juvenile dermatomyositis (JDM) and severe central nervous system (CNS) complications. All patients had at least 4 positive criteria of Bohan and Peter, which confirmed a definite diagnosis of JDM. They were all male, and had a relatively high creatinine kinase value at admission (1532-4260 U/l). Besides, progressive proximal muscle weakness and rash, one patient presented with rapid irreversible decline of vision. Ophthalmologic examination showed active vasculitis of the retina. After 2 weeks of treatment with immunosuppressive drugs and being in improved, relatively stable clinical condition, all 3 patients developed generalized tonic-clonic convulsions. Other causes of the neurological symptoms could be excluded. In all 3 patients, the course of JDM was fatal. The clinical symptoms and further investigations in our patients show CNS involvement in JDM. Although rarely reported, CNS vasculopathy can be a serious and life-threatening complication of JDM.
我们报告3例青少年皮肌炎（JDM）并伴有严重中枢神经系统（CNS）并发症的患者。所有患者均至少符合博汉和彼得标准中的4项阳性标准，从而确诊为JDM。他们均为男性，入院时肌酸激酶值相对较高（1532 - 4260 U/l）。此外，除了进行性近端肌无力和皮疹外，1例患者出现视力迅速不可逆下降。眼科检查显示视网膜有活动性血管炎。在接受免疫抑制药物治疗2周且临床状况有所改善并相对稳定后，所有3例患者均出现全身强直阵挛性惊厥。可排除神经症状的其他病因。所有3例患者的JDM病程均为致命性。我们患者的临床症状及进一步检查显示JDM存在中枢神经系统受累。尽管报道较少，但中枢神经系统血管病变可能是JDM的一种严重且危及生命的并发症。
