JUVENILE DERMATOMYOSITIS: CLINICAL CORRELATES OF VASCULAR DISEASE

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。假设：有青少年皮肌炎（JDM）病史的成年患者将有弥漫性血管病变的证据，其严重程度将与营养不良钙化的存在、TNF -308A等位基因和其他炎症指标以及心血管疾病的传统危险因素相关。本研究的目的是描述一组至少21岁且至少10年前被诊断为JDM的成年人的特征，包括：1)临床疾病活动评分(DAS)-总，皮肤和肌肉2)葡萄糖代谢：通过两次葡萄糖耐量试验测定胰岛素抵抗3)血管疾病：甲襞毛细血管研究，颈动脉内膜中膜厚度（CIMT）；4)炎症指标：TNF -308A、血清淀粉样蛋白和hsCRP 5)脂质代谢：空腹Lp（a)：胆固醇、HDL、LDL、LPAC、vsc、TGLM（中链甘油三酯）、ApoA1、ApoB、甘油三酯6）激素：DHEAS（男女）；LH， FSH，总睾酮（女性）

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. Hypothesis: 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 aim of this study is to characterize a cohort of adults, at least 21 years of age, and diagnosed with JDM at least ten years previously, with respect to: 1) Clinical Disease Activity Score (DAS)-total, skin and muscle 2) Glucose metabolism: to assay for insulin resistance by a two glucose tolerance test 3) Vascular disease: nailfold capillary studies, carotid intimal media thickness (CIMT); coronary calcium score by electron beam computerized tomography (EBCT) 4) Indicators of inflammation: TNF -308A, serum amyloid and hsCRP 5) Lipid metabolism: Fasting Lp(a): Cholesterol, HDL, LDL, LPAC, VLAC, TGLM (medium chain triglycerides), ApoA1, ApoB, triglycerides 6) Hormonal: DHEAS (males and females); LH, FSH, total testosterone (females)