Childhood-onset systemic lupus erythematosus: developing a blood test to help prevent heart disease in children most at risk

Childhood-onset systemic lupus erythematosus (cSLE), previously known as juvenile-onset systemic lupus, is a rare lifelong autoimmune condition which affects around 10,000 children and young people in the UK.[1] While treatments can help bring this illness under control, it can still cause serious long-term health risks that can shorten lives. Professor Coziana Ciurtin of University College London is developing a simple new blood test to identify children at increased risk of heart disease, a leading cause of early death in these patients. She hopes this could simplify risk assessments and pave the way for more targeted treatment, ultimately helping young people with cSLE live longer, healthier lives.

This project is jointly funded by Action Medical Research and LifeArc.

How are children’s lives affected now?

cSLE is a condition where the immune system mistakenly attacks healthy tissues, causing a range of symptoms and damage to vital organs, including the skin, joints, kidneys, nervous system, heart and lungs.

“cSLE affects each child differently, with symptoms ranging from very mild to life-threatening,” says Professor Ciurtin. “These may include fatigue, joint pain, hair loss and a distinctive ‘butterfly’ rash across their cheeks and nose, and in more severe cases, involvement of the kidneys, brain, lung, heart or liver.”

Although there is no cure, medications that suppress the immune system can control the disease, allowing many children to lead relatively normal lives. However, children and young people with cSLE remain at risk of serious long-term health complications – for example, they have a much greater likelihood of developing heart disease from early in life compared to their healthy peers.

“Heart disease is a major concern for many children with cSLE, caused by a build-up of fatty materials that narrows their arteries,” says Professor Ciurtin. “Unfortunately, it’s difficult to identify which children are most at risk – limiting the ability to target treatments to prevent this life-threatening complication.”

How could this research help?

“Our goal is to develop a blood test that can predict the risk of heart disease in children and young people with cSLE, improve their treatment and long-term outcomes,” says Professor Ciurtin.

Using data and samples from a large clinical trial in the US, the researchers previously identified blood markers that may be able to predict which children with cSLE are most likely to have thickened arteries.

“Building on these initial results, we now aim to confirm whether these markers can distinguish patients with cSLE with a higher risk of developing heart disease by testing them in another large group of children in the UK who will also have  their blood vessel  thickness measured,” says Professor Ciurtin.

The team then plans to develop a simpler, cheaper method for measuring these blood markers and assess its potential for routine clinical use.

“If successful, this blood test could identify children and young people with cSLE at higher risk of heart disease without having to scan their arteries,” says Professor Ciurtin. 

References

1. Peng J, et al. Atherosclerosis Progression in the APPLE Trial Can Be Predicted in Young People With Juvenile-Onset Systemic Lupus Erythematosus Using a Novel Lipid Metabolomic Signature. 2023;76(3):455-468.

We do not provide medical advice. If you would like more information about a condition or would like to talk to someone about your health, contact NHS Choices or speak to your GP.