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

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. © 2017 the author(s).