PT  - JOURNAL ARTICLE
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
TI  - Endothelial Activation Markers as Disease Activity and Damage Measures in Juvenile Dermatomyositis
AID  - 10.3899/jrheum.181275
DP  - 2019 Aug 01
TA  - The Journal of Rheumatology
PG  - jrheum.181275
4099  - http://www.jrheum.org/content/early/2020/01/10/jrheum.181275.short
4100  - http://www.jrheum.org/content/early/2020/01/10/jrheum.181275.full
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.