JUVENILE DERMATOMYOSITIS: CLINICAL CORRELATES OF VASCULAR DISEASE

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 有幼年型皮肌炎（JDM）病史的成年患者将有弥漫性血管病变的证据，其严重程度与营养不良性钙化的存在、TNF-308 A等位基因和其他炎症指标以及心血管疾病的传统风险因素相关。 本研究的具体目的是描述10-30名至少21岁且至少10年前诊断为JDM的成人的以下特征： a.临床疾病活动性评分（DAS）：总分、皮肤和肌肉 B.葡萄糖代谢：通过两次葡萄糖耐量试验检测胰岛素抵抗。 C.血管疾病：甲襞毛细血管研究、颈动脉内膜中层厚度（CIMT）、电子束计算机断层扫描（EBCT）冠状动脉钙化评分。 D.炎症指标：TNF-308 A、血清淀粉样蛋白和hsCRP。 e.脂质代谢：空腹Lp（a）：胆固醇、HDL、LDL、LPAC、VLAC、TGLM（中链甘油三酯）、ApoA 1、ApoB、甘油三酯。 F.激素：DHEAS（雄性和雌性）; LH、FSH、总睾酮（雌性）。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Adult patients with a history of Juvenile Dermatomyositis (JDM) will have evidence for diffuse vasculopathy, the severity of which will correlate with the presence of dystrophic calcification, with the TNF-308A allele and other measures of inflammation, and with traditional risk factors for cardiovascular disease. The specific aims of this study are to characterize 10-30 adults, at least 21 years of age, and diagnosed with JDM at least ten years previously, with respect to: a. Clinical Disease Activity Score (DAS): total, skin and muscle b. Glucose metabolism: to assay for insulin resistance by a two glucose tolerance tests. c. Vascular disease: nailfold capillary studies, carotid intimal media thickness (CIMT), coronary calcium score by electron beam computerized tomography (EBCT). d. Indicators of inflammation: TNF-308A, serum amyloid, and hsCRP. e. Lipid metabolism: Fasting Lp(a): Cholesterol, HDL, LDL, LPAC, VLAC, TGLM (medium chain triglycerides), ApoA1, ApoB, triglycerides. f. Hormonal: DHEAS (males and females); LH, FSH, total testosterone (females).