Juvenile idiopathic arthritis

Joint in pain

Juvenile idiopathic arthritis (JIA) is a persistent or recurring inflammation of the joints similar to rheumatoid arthritis, but in occurs in children, usually those below the age of 16. Though uncommon, it can be a crippling condition.

HIS fingers are crooked and gnarled, awkwardly bent at the joints. These hands are unable to hold a pencil or a spoon, or do up the buttons of a shirt.

These are not the hands of an elderly man. They belong to a seven year-old boy with juvenile idiopathic arthritis. But this seven year-old cannot copy notes in class, grip a badminton racket or tie his shoelaces.

Inflamed joints

Juvenile idiopathic arthritis (JIA) is persistent or recurring inflammation of the joints similar to rheumatoid arthritis in adults, but beginning before the age of 16,� says consultant rheumatologist Dr Chow Sook Khuan.

Children as young as six months can develop JIA, an uncommon disease that is believed to affect one in every thousand children around the world.

Doctors are still at a loss to explain what causes JIA, which is why the term idiopathic exists in the name (meaning the cause is unknown). However, we do know that it is not caused by an infection, eating the wrong types of food, events during pregnancy, injury or the weather.

JIA can affect children in different ways, for example the number of joints involved, and the symptoms can vary. This has given rise to three main classifications of JIA: pauciarticular JIA, polyarthritis and systemic disease (see table).

Western data shows that pauciarticular JIA is more common, forming up to 40-50% of all JIA cases. About 30% have polyarthritis and 10-15% have systemic disease,� says Dr Chow, although in practice, she sees more patients with the more severe forms of the disease.

Most children with JIA feel well, are fully active and have normal growth, although some may feel run down during flare-ups. More severe forms of the disease may cause problems with activities like walking, getting dressed, bathing and eating.

Juvenile idiopathic arthritis should be treated as early as possible to prevent joint deformities as seen in this picture.

Getting the diagnosis right

Diagnosis of JIA is a tricky thing, as there is no foolproof test and the symptoms are wide-ranging, fluctuating and vague.

Curiously, joint pain or swelling is not always the most obvious symptom, particularly in infants and very young children.

Fever, fever, fever! Dr Chow exclaims. Unexplained fever in a child and failure to grow, she says, are the main features that should alert parents and doctors.

Fever and rashes, often seen in systemic JIA, is a response from the immune system to the disease. Parents may also notice that their child stops growing or starts losing weight, although these features are not immediately obvious and will only be apparent after a few months.

The parents' or caregivers' observations of the child are crucial. An infant does not know how to explain (the symptoms), Dr Chow cautions, so parents need to keep their eyes peeled for unexplained behaviour, such as if the infant stops using a particular joint.

A baby may refuse to reach for the milk bottle, or a toddler may suddenly not want to get out of bed or walk, she describes.

Sometimes, blood tests are carried out to test for rheumatoid factor and antinuclear antibodies, which are present in some people with rheumatoid arthritis and related diseases. However, up to 80% of children with JIA do not have rheumatoid factor or antinuclear antibodies in their blood, while children with other conditions may have it.

What's the cure?

There is no cure for JIA, but there are ways to control the disease so that the symptoms will not limit the child's daily activities, or cause joint deformity and damage. This can only be achieved if proper treatment comes in as early as possible.

Dr Chow Sook Khuan...'Unexplained fever in a child and failure to grow should alert parents and doctors to the possibility of JIA.'

Polyarthritis and systemic disease have to be treated early and aggressively, Dr Chow stresses.

If you don't treat them early with special drugs like DMARDs (disease-modifying anti-rheumatic drugs), they will develop irreversible deformities and will need joint replacements. They may even develop life-threatening complications.�

DMARDs like methotrexate and sulphasalazine are very effective in controlling the progression of the disease. However, these drugs take several months to show any benefits.

Parents and doctors are worried about using DMARDs, as they are chemotherapy drugs, says Dr Chow. But if you delay using these drugs, joint deformities will set in.

Dr Chow assures parents that there is a lot of evidence to back the use of DMARDs in children with arthritis.

Other medications also have a role in controlling the symptoms of pain, stiffness and swelling. These include non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids.

Steroids are fast acting medications that are prescribed in low doses for a short period to control the inflammation. However, the child will not grow if you use too much steroids and do not know when to stop, cautions Dr Chow.

The good news is, most children will not grow up to have active adult arthritis. About two-thirds of children will outgrow JIA when they reach adulthood, says Dr Tang Swee Ping, consultant paediatrician and paediatric rheumatologist.

JIA usually follows a pattern where the disease flares up, then goes into remission, then flares up again. Some children are lucky, they may have only one or two flares. But some may have many flares, says Dr Tang.

On the other hand, there may also be children who just have chronic, low-grade activity for a prolonged period of time.

The ultimate aim of treatment is for JIA to go into remission, so that the child will be able to do everything that normal children can do, she says. But in reality, this can be a frustrating journey.

As is usually the case, up to four or five different types of medications and supplements are prescribed for an indeterminate period of time. The lack of an endpoint makes it difficult for children to weather it out.

A lot of patients have no problems taking the medication initially, but when they get older, they start refusing,� Dr Tang shares her experience.

She has a suggestion for parents: We compromise. We tell them, 'If you don't want to take all five medications, you take at least three. So you decide whether you want to take three or four today.'

This tactic makes the children feel like they have some sort of control, and compliance then becomes better.

Parents also need to realise that the treatment of JIA is a slow process, especially with the use of DMARDs that take several months to show any benefit.

A lot of parents end up frustrated, especially those whose children have difficulty controlling the disease. They will ask why the child is not getting any better, even though they keep bringing the child for treatment.

However, Dr Tang assures that although there is no cure, there is always hope that we can control the disease.

Note: Juvenile idiopathic arthritis is a relatively new term. Older terms used to describe this disease were juvenile chronic arthritis or juvenile rheumatoid arthritis.