O'Connor Kevin A, Abbott Kathy A, Sabin Brad, Kuroda Maxine, Pachman Lauren M
Molecular and Cellular Pathobiology Program, Children's Memorial Research Center, Box 212, Chicago, IL 60614, USA.
Clin Immunol. 2006 Sep;120(3):319-25. doi: 10.1016/j.clim.2006.05.011. Epub 2006 Jul 21.
Juvenile dermatomyositis (JDM), a systemic vasculopathy, is characterized by inflammation of skin and muscle. Muscle biopsies from untreated JDM patients show upregulation of type I interferon (IFN)-inducible genes, including myxovirus resistance protein A (MxA). The present study examines whether MxA mRNA expression in peripheral blood mononuclear cells (PBMC) from JDM patients: (1) is elevated compared to healthy controls, (2) reflects disease activity, and (3) changes with the onset of clinically effective treatment. MxA mRNA expression in JDM PBMC obtained at the initial clinic visit was elevated compared to controls and was positively correlated with Disease Activity Score (DAS) for muscle, but not with DAS for skin, suggesting that damage to skin and muscle in JDM may each have a discrete pathophysiology. During the course of clinically effective treatment, decrease in muscle symptoms was associated with a decrease in PBMC MxA mRNA expression.
青少年皮肌炎（JDM）是一种系统性血管病，其特征为皮肤和肌肉炎症。未经治疗的JDM患者的肌肉活检显示I型干扰素（IFN）诱导基因上调，包括抗黏液病毒蛋白A（MxA）。本研究探讨JDM患者外周血单个核细胞（PBMC）中MxA mRNA表达是否：（1）与健康对照相比升高；（2）反映疾病活动度；（3）随临床有效治疗的开始而变化。在初次门诊时获得的JDM患者PBMC中，MxA mRNA表达与对照相比升高，且与肌肉疾病活动评分（DAS）呈正相关，但与皮肤DAS不相关，这表明JDM中皮肤和肌肉损伤可能各自具有不同的病理生理学。在临床有效治疗过程中，肌肉症状的减轻与PBMC中MxA mRNA表达的降低相关。
