RT Journal Article
SR Electronic
T1 Endothelial Activation Markers as Disease Activity and Damage Measures in Juvenile Dermatomyositis
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP jrheum.181275
DO 10.3899/jrheum.181275
A1 Takayuki Kishi
A1 Jonathan Chipman
A1 Melvina Evereklian
A1 Khanh Nghiem
A1 Maryalice Stetler-Stevenson
A1 Margaret E.  Rick
A1 Michael Centola
A1 Frederick W.  Miller
A1 Lisa G.  Rider
YR 2019
UL http://www.jrheum.org/content/early/2019/07/23/jrheum.181275.abstract
AB 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.