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Premature Atherosclerosis in Juvenile Dermatomyositis

Children with juvenile dermatomyositis (JDM) may be at increased risk of early heart disease due to risk factors including high cholesterol, abnormalities with blood sugar, high blood pressure, limited physical activity, chronic inflammation and the medications that they take. In addition, patients with JDM often have inflammation in their blood vessels which may cause the blood vessels to function improperly. Researchers have now found that dysfunctional blood vessels may be one of the first signs of early heart disease. Although it is rare, involvement of the heart and heart attacks have been reported as causes of death in cases of children and adults with dermatomyositis.

This study will use a safe ultrasound technique to examine the function of the blood vessels in children with JDM. We will also look at risk factors to see if we can identify which ones may cause the greatest risk of early heart disease in these children. With the support of Cure JM and the CARRA network, we eventually hope to perform an additional study to look at these risk factors in a large population of children with JDM and to examine the ways that we can prevent heart disease in these young children.  

The long-term goal of our research is to identify which patients with JDM may be at highest risk of early heart disease and to learn if medications and dietary changes may help to prevent the development of early heart disease. In addition, the information that we gain from this study regarding the association between various risk factors and early heart disease will increase physician awareness and monitoring of these risk factors in children with JDM. Finally, by demonstrating that this safe ultrasound technique may be used as a measure of the very first signs of heart disease in children with JDM, we hope to provide physicians with a screening tool in pediatric patients with identifiable risk factors.

Study details:

Location

This study will be conducted at the Children’s Hospital at Montefiore, Bronx, NY

Principal Investigator

Dr. Dawn Wahezi, MD, MS

Contact #

(718) 696-2405

Eligible participants

Children, adolescents and young adults (age 2-21 years) with a diagnosis of JDM

Exclusion criteria

(1) diagnosis of a chronic illness (other than JDM) (2) current use of medications that alter lipid metabolism or endothelial function (including lipid lowering agents) and (3) current smoking.

Endopat testing

The patient will sit in a reclining chair with the hands at heart level and propped in a comfortable position. The Endo-PAT probe will be placed gently on the fingertip. Flexible tubing connects the finger probe to the machine. Baseline measurements will be recorded over 5 minutes. Then a blood pressure cuff will be inflated for 5 minutes, followed by rapid deflation. Measurements will additionally be recorded for at least 5 minutes after deflation. The patient’s other arm will serve as a control assessment. Prior evaluation of discomfort associated with this procedure resulted in a pain score of 1 on the Wong-Baker Faces Pain Scale.

Wong-Baker Scale