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Endothelial Activation Markers as Disease Activity and Damage Measures in Juvenile Dermatomyositis

Takayuki Kishi1,2, Jonathan Chipman1,2, Melvina Evereklian1,2

  • 1From the Environmental Autoimmunity Group, Clinical Research Branch, US National Institute of Environmental Health Sciences, National Institutes of Health (NIH); Coagulation Laboratory, NIH Clinical Center; Laboratories of Molecular Biology and Pathology, National Cancer Institute, NIH, Bethesda, Maryland; Oklahoma Medical Research Foundation; Haus Bioceuticals Inc., Oklahoma City, Oklahoma, USA.

The Journal of Rheumatology
|August 3, 2019
PubMed

Insights

Endothelial injury markers are elevated in juvenile dermatomyositis (JDM) patients and correlate with disease activity. Circulating endothelial progenitor cells (CEPC) are decreased, indicating impaired vascular repair in JDM.

Area of Science:

  • Vascular biology and immunology
  • Pediatric rheumatology

Background:

  • Endothelial dysfunction is implicated in various inflammatory conditions.
  • Circulating endothelial cells (CEC) and circulating endothelial progenitor cells (CEPC) are key indicators of endothelial health and vascular repair.
  • Juvenile dermatomyositis (JDM) is an autoimmune disease with potential vascular involvement.

Purpose of the Study:

  • To investigate markers of endothelial injury and repair in patients with JDM.
  • To correlate these endothelial markers with disease activity and damage in JDM.

Main Methods:

  • Assessed CEC, CEPC, von Willebrand factor (vWF) antigen and activity, factor VIII, P-selectin, and thrombomodulin in 20 JDM patients and controls.
  • Measured disease activity, damage, nailfold capillary density, and brachial artery flow-mediated dilation.
  • Analyzed serum cytokines/chemokines using Luminex assays.

Main Results:

  • JDM patients showed elevated CEC, vWF antigen, factor VIII, and thrombomodulin.
  • CEC correlated with pulmonary activity, while vWF antigen correlated with global, cutaneous, and extramuscular activity.
  • CEPC were negatively correlated with muscle activity, physical function, and endocrine damage.

Conclusions:

  • Patients with JDM exhibit increased markers of endothelial injury, linked to extramuscular disease activity.
  • Decreased CEPC in JDM patients suggest a disturbance in vascular repair mechanisms.
Abstract

Keywords:
CIRCULATING ENDOTHELIAL CELLSDISEASE ACTIVITYENDOTHELIAL FUNCTIONENDOTHELIAL PROGENITOR CELLSJUVENILE DERMATOMYOSITISMYOSITIS

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