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JUVENILE ARTHRITIS (STILLS DISEASE)

What is Juvenile Arthritis ?

Arthritis in childhood juvenile arthritis is very rare. There are many types and causes just as in adults. Diseases such as rheumatoid arthritis and ankylosing spondylitis also affect children, but they do not follow quite the same course as in adults. Arthritis is inflammation of joints. They become red, hot, swollen with fluid, and painful. The swollen joints are difficult to move or use. They are stiff in the mornings. The disease behaves differently in different children. In some it stays in a few joints only. In others it spreads to involve four or more joints: it is then referred to as polyarticular. One type of juvenile arthritis is notable for its high fevers, rash, pleurisy and swollen glands. This is called the systemic type. As well as the joint symptoms, some children also get a particular type of inflammation of the eye called anterior uveitis.

How does Juvenile Arthritis occur ?

The triggering factor for arthritis is not known. However, like rheumatoid arthritis it is probably a disorder of the immune (or body defence) system. Antibodies are chemicals produced in the blood against foreign agents such as viruses. In auto-immune disorders, the antibodies do not stop at killing these foreign agents: they can also damage the body's own tissues, such as the joints, the eyes and other organs like the skin and the lungs.

Why does Juvenile Arthritis occur ?

It is known that ankylosing spondylitis in children occurs particularly in older boys who have inherited the gene HLA B27, as in adults. No other inherited factor predisposes children to arthritis. It does not run in families.

Treatment Involved for Juvenile Arthritis

A painful, swollen, joint needs to be rested in bed with splinting and physiotherapy. Splinting involves supporting joints with specially made casts. This rests the joints. It also prevents them becoming distorted or deformed and keeps them in a good position for use. Physiotherapy will provide exercises to maintain the function of joints and strengthen muscles around them. Drug therapy consists of tablets (non-steroidal anti-inflammatory drugs, or NSAIDs) for relief of pain and swelling. There are over 40 types of NSAID. They can be taken regularly to begin with, and then reduced to be taken as and when needed. Steroids (cortisone-like drugs which dampen inflammation) will often be given for the systemic type of juvenile arthritis and inflammation of the eye but are used sparingly in other types of childhood arthritis. Special disease-modifying drugs such as methotrexate are used for severe cases. A new treatment called anti-TNF alpha has been developed for the treatment of juvenile arthritis. It has very complicated effects on the immune system, affecting both types of immune cells (B-cells and T-cells) as well as the chemical messages they send out to cause the inflammation. It is on trial and available in a number of specialist centres, and is given either subcutaneously (Etanercept) or intravenously (Infliximab). The drugs are expensive and therefore restricted, especially since they have to be given longterm. Initial results are very encouraging, although 5 year safety profiles are not yet available.

During Treatment for Juvenile Arthritis

The outlook is generally good in juvenile arthritis. Children often fare better than adults with similar joint disease. Anti-inflammatory drugs rarely cause side-effects such as rash or indigestion. Disease-modifying drugs can affect the blood cell count and this will be checked regularly and carefully. Steroid treatment in high dosage may result in a swelling of the face, a prominent abdomen, easy bruising, hair loss, and a stunting of growth. The steroid is therefore given in the lowest dose which is capable of controlling the disease. Steroid tablets are often taken on alternate days: this approach may reduce the side-effects.

After Treatment for Juvenile Arthritis

The outlook varies for the different types of juvenile arthritis. A few children with severe disease will need supervision into adulthood with physiotherapy and advice about dealing with deformed or fused joints. Those with eye involvement will have their eyes checked regularly over a long period. Very rarely amyloid disease occurs in long standing cases. Amyloid is an abnormal protein substance which results from the chronic active inflammatory process. This material may clog up certain tissues like the kidneys and liver, causing a variety of problems.

If Juvenile Arthritis is Left Untreated

Inflamed joints would heal with scar tissue fusing joints in abnormal positions. A few severely diseased children would end up in wheelchairs. Inflammation of the eye could lead to blindness. Amyloid disease would be more likely to occur.

Effects on Family of Juvenile Arthritis

The child with arthritis requires a lot of family support. Even with only a lower limb involved, attendance at school may be difficult. Children may have to attend regional centres in rheumatology to get expert management and expert care. Their psychological and educational development must be continued while their inflammation is controlled.