Systemic Juvenile Idiopathic Arthritis (sJIA) Fact Sheet
Contents
- 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?
What is Systemic Juvenile Idiopathic Arthritis (sJIA)?
Juvenile idiopathic arthritis (JIA) is a group of conditions characterised by chronicA disease of long duration generally involving slow changes. 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 autoimmuneAny condition caused by the body’s immune response against its own tissues. 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 polyarthritisInflammation in more than four joints. and approximately half of these children are left with significant disability
- It occurs in equal frequencyApplied to urination, needing to urinate more often than normal, for whatever reason. between boys and girls
What are the signs and symptoms of systemic JIA?
The main symptoms of JIA are swollen, painful and stiff joints. Additionally, systemic JIA has more severe symptoms, including:
- feverThe raising of the body temperature above norma, which may be accompanied by symptoms such as shivering, headache and sweating.
- rash
- tiredness and lack of energy
- swollen glands
- weight loss
Other characteristics of systemic JIA may include anaemiaA reduced level of haemoglobin, which carries oxygen in the blood. Anaemia causes tiredness, breathlessness and abnormally pale skin., enlargement of the liverA large abdominal organ that has many important roles including the production of bile and clotting factors, detoxification, and the metabolism of proteins, carbohydrates and fats. and/or spleenAn organ situated on the left side of the abdomen that filters out worn-out red blood cells and other foreign bodies from the bloodstream., inflammationThe body’s response to injury. of the lining of the heart and/or lungs4 and stunted growth.
What is the burden of systemic JIA?
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 visualRelating to the sense of sight (vision). outcome of children and impacts functioning in school and home.
What is the prognosis for systemic JIA?
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.
How is systemic JIA diagnosed?
No specific laboratory test can diagnose sJIA, but bloodA fluid that transports oxygen and other substances through the body, made up of blood cells suspended in a liquid. tests check for the presence of autoantibodies and haemoglobinThe oxygen carrying pigment that is present in red blood cells. levels, and X-rays, MRIAn abbreviation for magnetic resonance imaging, a technique for imaging the body that uses electromagnetic waves and a strong magnetic field., CTThe abbreviation for computed tomography, a scan that generates a series of cross-sectional x-ray images or ultrasound scanThe process of using high-frequency sound waves to produce internal images of the body. may be used to check for any signs of inflammation in the joints or fluid build-up around the heart or lungs.
What treatment options are available?
Mild forms of disease are usually treated with non-steroidal anti-inflammatory drugsA group of drugs that give pain relief and reduce inflammation. such as ibuprofen. High dose corticosteroidsA group of hormones that are produced by the adrenal glands, which sit on top of the kidneys. 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 factorA protein that stimulates inflammation and causes cells to die. (TNFAn abbreviation for tumour necrosis factor, a protein that stimulates inflammation and causes cells to die.) therapy.
