Endothelial progenitor cell number is not decreased in 34 children with Juvenile Dermatomyositis: A pilot study

Dong Xu, Akadia Kacha-Ochana, Gabrielle A. Morgan, Chiang Ching Huang, Lauren M. Pachman*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

7 Scopus citations


Objective: A pilot study to determine endothelial progenitor cells (EPC) number in children with Juvenile Dermatomyositis (JDM). Methods: After obtaining informed consent, the EPC number from 34 fasting children with definite/probable JDM at various stages of therapy-initially untreated, active disease on medication and clinically inactive, off medication-was compared with 13 healthy fasting pediatric controls. The EPC number was determined by fluorescence activated cell sorting (FACS), CD34+/VEGFR2+/CD45dim-, and assessed in conjunction with clinical variables: disease activity scores (DAS), duration of untreated disease (DUD), TNF-α allelic polymorphism (A/G) at the promoter region of -308, number of nailfold capillary end row loop (ERL) and von Willebrand factor antigen (vWF:Ag). Correlations of the EPC numbers with the clinical and demographic variables, including DAS Skin (DAS SK), DAS Weakness (DAS WK), DAS Total Score, DUD, Cholesterol, triglycerides, High-Density Lipoprotein (HDL) and Low-Density Lipoprotein (LDL), and ERL were calculated using the Pearson correlation coefficient. Tests of associations of EPC with gender (boy vs girl), TNF-α-308A allele (GA/AA vs GG), vWF:Ag (categorized by specific ABO type) as normal/abnormal were performed, using two-sample T- tests. Results: The EPC number for JDM was not significantly different from the healthy controls and was not associated with any of the clinical or cardiovascular risk factors tested. Conclusion: The EPC for JDM were in the normal range, similar to adults with DM. These data support the concept that the normal EPC numbers in DM/JDM, irrespective of age, differs from adult PM, where they are decreased, perhaps reflecting a different pathophysiology.

Original languageEnglish (US)
Article number42
JournalPediatric Rheumatology
Issue number1
StatePublished - May 17 2017


  • Endothelial Progenitor Cell (EPC)
  • Fluorescence Activated Cell Sorting (FACS)
  • Juvenile Dermatomyositis (JDM)

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Rheumatology
  • Immunology and Allergy


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