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.
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.
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.
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.
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.
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.
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.
References / further reading:
Midgley A, Watson, L and Beresford, MW (2014) New insights into the pathogenesis and management of lupus in children. Arch Dis Child 2014; 0:1–5. doi:10.1136/archdischild-2013-304397.
- Watson, L., V. Leone, C. Pilkington, et al. Juvenile-onset SLE; disease activity, severity and damage - the UK JSLE Cohort Study. Arthritis Rheum, 2012.
- Tucker, L.B., A.G. Uribe, M. Fernandez, et al. Adolescent onset of lupus results in more aggressive disease and worse outcomes: results of a nested matched case-control study within LUMINA, a multiethnic US cohort (LUMINA LVII). Lupus, 2008. 17(4): p. 314-22.
- Maidhof, W. and O. Hilas. Lupus: an overview of the disease and management options. P T, 2012. 37(4): p. 240-9.
- Ardoin, S.P. and L.E. Schanberg. The management of pediatric systemic lupus erythematosus. Nat Clin Pract Rheumatol, 2005. 1(2): p. 82-92.
- Livingston, B., A. Bonner, and J. Pope. Differences in clinical manifestations between childhood-onset lupus and adult-onset lupus: a meta-analysis. Lupus, 2011. 20(13): p. 1345-1355.
- Sato, V.A., I.D. Marques, P.T. Goldenstein, et al. Lupus nephritis is more severe in children and adolescents than in older adults. Lupus, 2012.
- Cairoli, E., M. Rebella, N. Danese, et al. Hydroxychloroquine reduces low-density lipoprotein cholesterol levels in systemic lupus erythematosus: a longitudinal evaluation of the lipid-lowering effect. Lupus, 2012.
- Kazyra, I., C. Pilkington, S.D. Marks, et al. Mycophenolate mofetil treatment in children and adolescents with lupus. Arch Dis Child, 2010. 95(12): p. 1059-61.
- Podolskaya, A., M. Stadermann, C. Pilkington, et al. B cell depletion therapy for 19 patients with refractory systemic lupus erythematosus. Arch Dis Child, 2008. 93(5): p. 401-6.