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 1011
OP 1018
DO 10.3899/jrheum.181275
VO 47
IS 7
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 2020
UL http://www.jrheum.org/content/47/7/1011.abstract
AB Objective. Circulating endothelial cells (CEC), von Willebrand factor (vWF) antigen, P-selectin, and thrombomodulin are released from damaged endothelium, while decreases in circulating endothelial progenitor cells (CEPC) have been associated with poor vascular outcomes. We examined these markers in the peripheral blood of patients with juvenile dermatomyositis (JDM) and their correlations with disease assessments.Methods. Peripheral blood endothelial cells and biomarkers were assessed in 20 patients with JDM and matched healthy controls. CEC and CEPC were measured 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 density, and brachial artery flow dilation were assessed. Serum cytokines/chemokines were measured by Luminex.Results. CEC, vWF antigen, factor VIII, and thrombomodulin, but not vWF activity, CEPC, or P-selectin, were elevated in the peripheral blood of patients with JDM. CEC correlated with pulmonary activity (rs = 0.56). The vWF antigen correlated with Patient’s/Parent’s Global, cutaneous, and extramuscular activity (rs = 0.47–0.54). CEPC negatively correlated with muscle activity and physical function (rs = −0.52 to −0.53). CEPC correlated inversely with endocrine damage. The vWF antigen and activity correlated with interleukin 10 and interferon-gamma inducible protein-10 (rs = 0.64–0.82).Conclusion. Markers of endothelial injury are increased in patients with JDM and correlate with extramuscular activity. CEPC correlate inversely with muscle activity, suggesting a functional disturbance in repair mechanisms.