JUVENILE DERMATOMYOSITIS: CLINICAL CORRELATES OF VASCULAR DISEASE

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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)