TY - JOUR T1 - Endothelial Activation Markers as Disease Activity and Damage Measures in Juvenile Dermatomyositis JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.181275 SP - jrheum.181275 AU - Takayuki Kishi AU - Jonathan Chipman AU - Melvina Evereklian AU - Khanh Nghiem AU - Maryalice Stetler-Stevenson AU - Margaret E. Rick AU - Michael Centola AU - Frederick W. Miller AU - Lisa G. Rider Y1 - 2019/08/01 UR - http://www.jrheum.org/content/early/2019/07/23/jrheum.181275.abstract N2 - Objective Circulating endothelial cells (CECs), von Willebrand Factor (VWF) antigen, P-selectin and thrombomodulin are released from damaged endothelium, while decreases in circulating endothelial progenitor cells (CEPCs) have been associated with poor vascular outcomes. We examined these markers in the peripheral blood of juvenile dermatomyositis (JDM) patients and their correlations with disease assessments. Methods Peripheral blood endothelial cells and biomarkers were assessed in 20 JDM patients, and matched healthy controls. CECs and CEPCs were quantitated by flow cytometry, while VWF antigen and activity, Factor VIII, P-selectin and thrombomodulin were measured in plate-based assays. Disease activity and damage, nailfold capillary (NFC) density, and brachial artery flow dilation were assessed. Serum cytokines/chemokines were measured by Luminex. Results CECs, VWF antigen, Factor VIII, thrombomodulin, but not VWF activity, CEPCs or P-selectin, were elevated in the peripheral blood of JDM patients. CECs correlated with pulmonary activity (rs= 0.56). VWF antigen correlated with Patient/Parent Global, cutaneous and Extra-muscular Activity (rs= 0.47-0.59). CEPCs negatively correlated with muscle activity and physical function (rs= -0.52- -0.53). CEPCs correlated inversely with endocrine damage. VWF antigen and activity correlated with IL-10 and IP-10 (rs= 0.64-0.82),. Conclusion Markers of endothelial injury are increased in JDM patients and correlate with extramuscular activity. CEPCs correlate inversely with muscle activity, suggesting a functional disturbance in repair mechanisms. ER -