This article presents an overview of the most serious type of childhood arthritis, systemic juvenile idiopathic arthritis, and discusses the symptoms, prognosis and various treatment options.
- What is Systemic Juvenile Idiopathic Arthritis (sJIA)?
- What are the signs and symptoms of systemic JIA?
- What is the burden of systemic JIA?
- What is the prognosis for systemic JIA?
- How is systemic JIA diagnosed?
- What treatment options are available?
Juvenile idiopathic arthritis (JIA) is a group of conditions characterised by chronic arthritis in children. Systemic JIA is the subset of JIA with the worst long-term prognosis and is the most serious type of childhood arthritis.
- It is estimated that JIA develops in about a thousand children in the UK every year; its cause isn't fully understood at present but it's thought to be an autoimmune disease
- Systemic JIA is a type of JIA with the worst long-term prognosis, representing 10-20% of all juvenile idiopathic arthritides
- Systemic JIA affects up to 2,500 children under 16 in the UK
- Systemic JIA begins in children aged 16 years or younger
- The peak age of onset is between 18 months and two years
- One-half to two-thirds of patients develop a chronic persistent polyarthritis and approximately half of these children are left with significant disability
- It occurs in equal frequency between boys and girls
The main symptoms of JIA are swollen, painful and stiff joints. Additionally, systemic JIA has more severe symptoms, including:
- tiredness and lack of energy
- swollen glands
- weight loss
Other characteristics of systemic JIA may include anaemia, enlargement of the liver and/or spleen, inflammation of the lining of the heart and/or lungs4 and stunted growth.
The only data available, for JIA, suggest the disease imposes a significant burden on different spheres of the patients', caregivers' and family's life. It also imposes a societal burden of significant health care costs and utilisation, affecting quality of life, physical function and visual outcome of children and impacts functioning in school and home.
Some children may be free of disease within two to four years, whereas others experience a relapsing course with both systemic and arthritic symptoms; the remaining patients can have persistent arthritis.
No specific laboratory test can diagnose sJIA, but blood tests check for the presence of autoantibodies and haemoglobin levels, and X-rays, MRI, CT or ultrasound scan may be used to check for any signs of inflammation in the joints or fluid build-up around the heart or lungs.
Mild forms of disease are usually treated with non-steroidal anti-inflammatory drugs such as ibuprofen. High dose corticosteroids are used to control systemic symptoms, but do not improve the long-term prognosis and can cause severe side effects. Treatment with traditional arthritis medications such as methotrexate is of little benefit and only 30 percent of children may respond to anti-tumour necrosis factor (TNF) therapy.