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Systemic lupus erythematosus – helping children and teenagers understand why they are affected by this rare and disabling condition

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What did the project achieve?

“Our work has increased understanding of why young females are at a greater risk of developing systemic lupus erythematosus (SLE) than young males,” says Dr Kate Webb of University College London. “We also identified potential new leads for the development of effective new treatments that can help reduce the long-term impact of this distressing illness on children’s lives.”

SLE is a rare, unpredictable condition that is caused when a person’s immune system attacks their body tissues. The symptoms will vary between affected individuals, but can include skin rash, fever and fatigue. When the illness starts in childhood, it most commonly affects girls around 11 or 12 years of age.1 Sadly, there is no cure – and it tends to be most severe if it starts during childhood, sometimes proving life-threatening.

This research involved more than 100 males and females aged between six and 18 years of age. The team developed sophisticated new blood separation techniques (including one used for the first time in the UK) to look at specific cells and molecules involved in the immune response before, during and after puberty – searching for clues that may help explain why SLE is more common in young women after puberty.

“We found that all young people make more of a key molecule called interferon-alpha – which is produced by certain immune cells – after puberty, regardless of their sex,” says Dr Webb. “But young women produce even higher amounts of this molecule than young men – putting them at greater risk of developing conditions like SLE.”

The team also identified other parts of the immune system that could also be involved in the development of SLE – generating new knowledge that could potentially help to develop new treatments in the future.

“This study helps to explain to young people with SLE why they may have developed the condition – and how this is linked to sex and puberty,” says Dr Webb. “It also highlights the importance of interferon-alpha in the development of the illness – and identifies new avenues for future research.”

This research was completed on

Research Training Fellowship*: Dr Kate R Webb

Children with a disabling, rare condition called systemic lupus erythematosus (SLE) may one day benefit from research by Dr Kate Webb of University College London. When this distressing disease starts during childhood, it most typically affects girls of around 11 or 12 years of age.1 Their symptoms vary, but can include skin rashes, fever and fatigue. Sadly, there’s no cure and gaps in understanding make it impossible to answer some important questions that children ask, such as ‘Why me?’, ‘Why now?’ and ‘How will my life be affected?’. Dr Webb hopes her research will help put that right.

How are children’s lives affected now?

“The way in which SLE affects each individual child is different, but symptoms can range from skin rashes, arthritis and low blood counts, to kidney problems, headaches and seizures,” says Dr Webb.

There’s no cure for SLE. Once children have developed the disease, they will have it for the rest of their lives.

“Medications for SLE can ease symptoms,” says Dr Webb. “Unfortunately, though, they often cause side effects, such as weight gain, tiredness and increased susceptibility to infections. Some medication can also affect fertility.”

SLE is unpredictable, causing varying levels of disability. Sadly, it tends to be most severe if it starts during childhood, sometimes proving life threatening.

“Young people with SLE want to know why they’ve got it, why it has started while they’re so young, and how it will affect their lives,” continues Dr Webb. “Unfortunately, it’s impossible to answer these questions properly, which is highly frustrating for everyone concerned.”

 

How could this research help?

“We know that SLE is more common in girls, and that it often starts during puberty, but we don’t know why,” says Dr Webb. “I am trying to help fill that gap in understanding. Doing so could help answer common questions that young people ask when they develop SLE, such as ‘Why me?’ and ‘Why now?’.”

The symptoms of SLE result from the immune system mistakenly attacking parts of body – such as the skin, blood, brain, joints, kidneys, lungs and heart. Dr Webb is investigating possible reasons for this attack during puberty, by studying how hormonal changes affect the immune system.

“By increasing our understanding of the disease processes involved in SLE, my work could help us to identify possible new approaches to treatment,” says Dr Webb.

Estimates suggest hundreds of children are living with SLE in the UK.2 The need for this research is high.

References

1. Levy DM et al. Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am 2012; 59: 345–364.

2. Arthritis Research UK. Lupus. http://www.arthritisresearchuk.org/arthritis-information/data-and-statis... Website accessed 10 March 2015.

 

* Research Training Fellowships:
Each year, Action Medical Research awards these prestigious grants to help the brightest and best doctors and scientists develop their career in medical research

 

Project Leader Dr Kate R Webb MBBCh MMed FCPaed (SA) Cert Rheum (SA)
Project Team Dr John Y Ioannou PhD FRCPProfessor Gary Butler MD FRCPCHProfessor Lucy Wedderburn PhD FRCP
Project Location Division of Medicine, Centre for Adolescent Rheumatology, University College London
Project Location Other Paediatric and Adolescent Medicine and Endocrinology, Institute of Child Health, University College London
Project duration 3 years
Date awarded 5 February 2015
Project start date 1 June 2015
Project end date 31 January 2019
Grant amount £206,806
Grant code GN2357

 

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