Vascular Injury and Dysfunction in Pediatric Lupus
Vascular Injury and Dysfunction in Pediatric Lupus

November 3, 2020

While children with lupus can experience manifestations similar to adults with the disease, children present with more acute illness, more frequent flares, and increased risk of major organ involvement—including those of the heart.

With her LRA grant, Joyce Chang, MD, MSCE from Children’s Hospital of Philadelphia, aims to understand why children with lupus are more prone to heart attacks and strokes—and how to prevent these conditions from happening in the first place.

We currently do not know how to monitor or prevent early blood vessel injury in child-onset lupus,” said Dr. Chang. “My investigation will examine whether a specific pathway is involved in the initial injury that causes vascular dysfunction and premature plaque formation.”

Specifically, Dr. Chang is looking at the role of neutrophils—the most abundant immune cell type—and their role in the initiation and perpetuation of tissue damage to the heart. “So, it’s possible that there’s a sort of mechanistic link between neutrophil-induced inflammation in lupus and some of the accelerated plaque formation that we see,” explained Dr. Chang.

As a researcher and a clinician, I enjoy taking care of my pediatric lupus patients, most of who are teenagers. Adolescence can be a time of great personal growth – so to take care of them is especially rewarding.” — Dr. Joyce Chang

Understanding that mechanism and how lupus can cause injury to blood vessels—specific to children and adolescents—will allow Dr. Chang and her team to make two important advances. They will be able to quantify factors to determine a patient’s lupus-related risk of having a heart attack or stroke. And they aim to determine potential ways to intervene and inhibit neutrophils activation or some form of its downstream effects.

Because neutrophils play a major role in fighting infection, we need to find their specific attribute that causes blood vessel injury,” added Dr. Chang. “Then we can target that attribute rather than trying to wipe them out completely.”

The LRA recognizes the importance of Dr. Chang’s work because there are health care disparities with lupus—and especially pediatric lupus. Likewise, Dr. Chang acknowledges the role the LRA plays: “The LRA really drives scientific lupus research—and without that support, many of the studies of early investigators, like mine, might never get off the ground. I’m deeply grateful for this opportunity.”

Dr. Chang’s investigation has the potential to support future studies that could lead the way to therapies that are beneficial in supporting cardiovascular health in adolescents and young adults with lupus.

As a researcher and a clinician, I enjoy taking care of my pediatric lupus patients, most of who are teenagers. Adolescence can be a time of great personal growth – so to take care of them is especially rewarding.” — Dr. Joyce Chang

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