UK JSLE Study Group

Juvenile-onset Systemic Lupus Erythematosus (Childhood lupus) is a multi-system, autoimmune disorder with a wide range of clinical presentations. SLE is often referred to as ‘lupus’.

Despite advances in our understanding of SLE, the cause remains unknown. Both genetic and environmental factors appear to lead to over-activation of parts of the body’s own immune system. This leads to the immune system attacking different parts of the body, leading to the symptoms of SLE. SLE is uncommon in adults and even more uncommon in children. About 15 – 20% of patients develop SLE in childhood or adolescence.

When SLE presents in childhood or adolescence it is called juvenile-onset SLE (JSLE) or ‘childhood lupus’. JSLE differs in some important aspects from the disease in adults. Childhood-onset disease is generally more severe than adult-onset disease. Survival rates from JSLE have improved. However,there is still a significant associated morbidity or burden from the disease or treatments used to treat children. This underlines the need for a better understanding of this condition and better therapies.

The rarity of JSLE (affecting about 60-300 children in a million each year in the UK) makes it very difficult to carry out research into the condition. This website gives information about the work of the UK JSLE Study Group, including information for patients, parents and health professionals. 

In 2006 a group of leading UK Paediatric Rheumatologists, Nephrologists and other specialists formed the UK JSLE Study Group. The UK JSLE Study Group is a multi-disciplinary group of paediatric rheumatologists, nephrologists and dermatologists, adult rheumatologists, nurse specialists, lay representatives and basic scientists from all over the UK. Its members represent almost all of the major paediatric centres in the UK. Its aims are to develop a comprehensive research program to investigate the "Clinical characteristics and immunopathology of JSLE".

The group set up the UK JSLE Cohort Study and Repository in 2006.The study regularly collects clinical data from patients participating in the study as well as biological specimens such as blood and urine. The data and specimens facilitate lots of research into JSLE. Please see the Cohort Study section for more information.

The resource is open to collaborative project applications from other researchers who can apply to conduct research using the existing Cohort data.

Visit our research pages

What is JSLE?

Juvenile Onset Systemic Lupus Erythematosus (JSLE) (commonly referred to as Lupus or Childhood Lupus) is a chronic autoimmune disease in children that can affect any part of the body including skin, joints and of the major organs. Depending on ethnicity, the incidence of JSLE is said to be 0.4 – 0.9 per 100,000 children per year. Between 15-20% of lupus patients develop the disease in childhood.

JSLE can be very variable in how it manifests, with some children having a mild disease and others having a very severe disease. The course of JSLE is characterised by episodes of disease flares, followed if treated promptly and appropriately, by periods of improvement. Generally, children require more intensive drug therapy than is needed for adult patients.

The main characteristic in the immunology underlying lupus is the development of auto-antibodies directed against the person’s own cell’s nuclear proteins (i.e. nuclear auto-antigens), especially double stranded DNA.

Symptoms

Often JSLE can present with a broad range of non-specific symptoms (such as mouth ulcers, weight loss, headache, and joint pains), which can also be common, yet entirely normal symptoms of childhood and adolescents.  Children with JSLE may suffer with myalgia, arthralgia or arthritis, and inflammation of the kidneys, lungs and brain. Severe unremitting fatigue is a very frequent and often disabling symptom. Skin rashes, headaches / migraines, depression, light sensitivity, difficulty with memory / concentration and circulation problems are common experiences.  Many of these symptoms are known to have a detrimental impact on health related quality of life and well-being including education.

Diagnosis

Making a diagnosis of JSLE has an enormous impact on the child or young person and their family and can affect many aspects of their personal, educational and physical development. 

A diagnosis of JSLE can made by using the American College of Rheumatology (ACR) classification criteria.  Four out of the 11 criteria (which include rash, arthritis and renal disorder), not necessarily at the same time point, are highly suggestive of a diagnosis of JSLE, although sometimes in childhood, a diagnosis of probable lupus will also be made without strictly meeting four or more criteria. Other classification systems for lupus in children are being assessed.

Blood tests help in forming a diagnosis of JSLE and in on-going disease monitoring. They can be very helpful in anticipating or interpreting a disease flare, and in ensuring resolution of it. They are used alongside detailed clinical examination that should always be comprehensive and full, at every clinic visit. A detailed set of investigations will also be needed, depending on the extent and nature of the child’s disease.

Management

The treatment of JSLE is individualised and based on the extent of organ involvement and severity of disease.  The goal of treatment is to prevent  permanent organ damage and maximise disease control.  An experienced multi-disciplinary team of healthcare professionals should look after children and young people with lupus familiar with the complexity of lupus in childhood and adolescence.

A range of medications can be used depending on the child’s symptoms. Non-steroidal anti-inflammatory drugs (NSAIDs) can be used for some symptomatic relief, provided that the child does not have renal impairment.  However the main focus of treatment is to control the underlying disease process. Corticosteroids are used in most children, especially in the early phase of the disease. A range of immunosuppressive drugs including anti-malarials and disease modifying drugs such as azathioprine, mycophenolate mofetil, cyclophosphamide can be used to treat JSLE. Newer treatments are also available and being assessed for use in children with lupus. 

All children with JSLE are encouraged to wear sun-block since ultraviolet (UV) light can act as a trigger for disease flares. Artificial lighting can sometimes be problematic for lupus patients. All children and their families are also encouraged to have the flu vaccine annually. This helps to reduce the number and severity of flu episodes that children with JSLE may have over the year.

Disease flares

JSLE is a relapsing and remitting condition.  Specific disease activity indices have been developed for lupus to assess these.  Regular monitoring of symptoms / disease activity along with blood and urine tests are fundamental in checking the progress of the condition. Disease relapses require thorough assessment and investigation followed by treatment according to the severity of organ involvement.  Mild flares may respond to a dose increase of therapy or short term increase of steroids.  Moderate to severe flares are likely to need intravenous steroid therapy to achieve remission and may need further immunosuppressant treatment.  Prompt recognition, intervention and appropriate treatment of disease flares is important as disease damage can occur during periods of poor control.

Further information

More information is available from local Paediatric Rheumatology teams or from the LUPUS UK website (www.lupusuk.org.uk). LUPUS UK is a national charity for people with lupus that supports the whole family to help them learn about lupus and how they can adjust to living well with the illness. The charity also raises money to produce information about lupus,  fund specialist nurses and support lupus research.

Conclusion

JSLE is a complicated disease, which requires specialist multi-disciplinary teams to assess, treat and monitor.  Aggressive treatment regimens are usually required.  The multi-disciplinary paediatric rheumatology teams usually leads the management of these patients, working in close partnership with all health professionals involved in their care including school nurses who can play an important role.  

Contributing Centres