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Juvenile Idiopathic Arthritis

Introduction

It must be a big shock to be told that your child has arthritis because everyone thinks that arthritis only happens to old people. It will take sometime before your family and friends can understand that actually children and young people too can have a form of arthritis which the doctors call ‘Juvenile Idiopathic Arthritis’. This information leaflet will answer some of the questions that you may have and if you have any other questions, write it down and ask your doctor.

What is Juvenile Idiopathic Arthritis (JIA)?

JIA stands for Juvenile Idiopathic Arthritis. Juvenile means that the arthritis starts in children and young persons before the age of 16 years. Idiopathic means that we still do not know what causes this disease. Arthritis means that one or more than one of your joints are inflamed – that is they are swollen, painful, stiff and he/she may not be able to move them as normal. And the arthritis has been present for longer than 6 weeks.

JIA is a relatively new term and previously, older terms were used by doctors and these included Juvenile Chronic Arthritis (JCA) or Juvenile Rheumatoid Arthritis (JRA). However, doctors today prefer to use the term JIA.

What causes JIA ?

The exact cause of JIA is still not known. There would appear to be a number of factors that seem significant but these are still being actively studied. There is no clear evidence that it is inherited (i.e. run in families) but we know that some genetic markers are found more frequently in certain kinds of arthritis in children and adults.

We know that:

It is not caused by an infection
It is not caused by eating the wrong types of food
It is not caused by events that occurred during pregnancy
It is not caused by trauma or injury
It is not caused by certain weather (Although some people report that their arthritis gets better in certain weather, there is no clear evidence)  

How does arthritis happen ?

In a healthy person, a process called ‘inflammation’ can occur and which is normal. Inflammation is an essential response of the immune system of a healthy person to fight foreign invaders (e.g. viruses or bacteria) or to respond to tissue injury. After the injury has healed or the foreign invader eliminated, the immune system “turns off” the inflammatory response, and the inflammation goes away.

In JIA, the immune system does not function normally and it directs an inflammatory response against the body’s own healthy tissues rather than against infection. In addition, once started, the immune system does not ‘turn off’ as normally. We do not know what triggers the unusual inflammation or why the inflammation cannot be stopped by normal mechanisms. And it is this ongoing inflammation that results in arthritis. Due to this abnormal response of the immune system, JIA is also referred to as an ‘autoimmune disease’, that is one’s immune system is directed against oneself.

Who gets arthritis in childhood ?

Any child or young person can get arthritis. If can affect both girls and boys although in general, about 70% of children who get arthritis are girls. It can also affect a child of any age although they are most common between the ages of 2 to 6 years. There are various different types of JIA and each of this has a different pattern in which they may affect different ages or sexes more. For e.g. Enthesitis related arthritis (ERA) affects older and teenage boys more than girls. Polyarthritis usually affects girls more and systemic onset arthritis can affect both girls and boys in an equal manner.

Is arthritis common ?

We do not have any statistics for Malaysia as yet. It is not a very common disease like asthma or pneumonia or diarrhoea. In most regions around the world, one in every thousand children has some form of ongoing arthritis at any one time.

What are the symptoms of JIA ?

The commonest symptoms are joint pain, joint stiffness and joint swelling.

Joint pains are usually not very severe and most often just mild to moderate in severity. The pain is commonly more prominent on waking up and eases as the day goes by or with exercise. However, some children especially the very young may not complain of any joint pains at all because they might not recognize that this is not the way it is supposed to be. In addition, some older children may have swollen joints but do not suffer from much pain. Therefore, not every child who has joint pains has arthritis, and similarly not every child with arthritis has joint pains.

Children and young people can also suffer from joint stiffness like elderly people. Joint stiffness occurs especially after periods of inactivity e.g. on waking up in the morning, after a long afternoon nap or after a long journey in the car. You may have difficulty walking if your lower limbs joints are affected and you might seem to ‘walk in a funny manner, usually like a ‘robot’. If your fingers or wrists are involved, you might have difficulty brushing your teeth or opening taps. The stiffness usually improves with exercise and may last for as short as a few minutes or as long as a few hours.

Your joints can also be swollen and the joint swelling may vary from being mild to severe. The severity of joint swelling may fluctuate from day to day (some days more and some days less) but is generally present if untreated. When your joints are very swollen, you will tend to keep them bent as this is the most comfortable position to be in, but this is not the best position for your joints to be in as they can become stuck in this bent position (develop contractures). Sometimes the swollen joint may also feel warm but it is unusual for the overlying skin to become inflamed (red).

Some children and young people who have a particular type of arthritis called ‘systemic JIA’ (see below) might have other symptoms like a high fever on and off for weeks, feeling generally unwell and lethargic, and a red rash which comes and goes (usually appears when the temperature if high and subsides when the temperature settles).

Other children may sometimes be suspected of having arthritis due to problems like a limping gait, or a delay or regression of their developmental milestones(e.g. a toddler who has started to walk, is now refusing to walk but has gone back to crawling). Sometimes a child with arthritis might not use a limb that is affected.

Are all joint pains due to arthritis ?

No, not all joint pains are due to arthritis and not all forms of arthritis are Juvenile Idiopathic Arthritis. In fact, in children and young people, arthritis is an uncommon cause of joint pain. There are many other causes of joint pains and your doctor will be able to confirm if you have arthritis.

How is JIA diagnosed ?

It is usually quite difficult to diagnose arthritis as there is no foolproof test. It is also a rare disease and the symptoms are wide ranging, fluctuating and although seem serious, are often vague. For many, this may mean a delay in diagnosis and many tests need to be carried out before doctors can be certain.

Your doctor will ask you a series of questions, examine your joints and also do some tests to arrive at a diagnosis. Often your doctor needs to do these tests to rule out other conditions that may cause joint pains but are not arthritis.

These tests which may be done include:

What are the different types of JIA ?

JIA is an big term that covers many different forms of arthritis in children. While every child who has a chronic arthritis which is not due to other rheumatic diseases has Juvenile Idiopathic Arthritis, it is very clear that every child with JIA does not have the same disease. It is important to recognize the different forms of arthritis because they have a different prognosis (probably outcome in the future), different response to medications and most likely a different course. They different types are:

Oligoarthritis

This is the most common form of arthritis and usually affects children less than the age of 5 years. It affects more girls than boys. It only affects a few joints (4 or less) in the first 6 months of disease and most commonly affects the knees or ankles. In majority of the patients, they only have one joint involved. This is the mildest form of arthritis and has the best long term outcome. However this type of arthritis has the highest chance of developing uveitis (inflammation of the eye) which may be silent and will not cause symptoms like eye pain, redness or changes in vision. Uveitis can be treated if diagnosed but if undiagnosed early, permanent vision loss or blindness can occur.

Extended Oligoarthritis

In this type of arthritis, you may appear to have few joints affected (oligoarthritis) in the first six months, but this progresses to involve more joints (5 or more) after that i.e. become polyarthritis. This type of JIA can cause damage to your joints and may require stronger medicines.

Polyarthritis

This is the next most common type of arthritis in children. Polyarthritis means that many joints are inflamed, usually 5 or more in the first 6 months of disease. This type of arthritis tends to affect girls more. Except for joint inflammation, these children do not usually appear particularly ill. High fevers and skin rash are not seen, although you may have mild anemia, loss of appetite and decreased overall growth.

In the form of arthritis, the joints are usually involved in a symmetrical manner meaning that both the joints of the right and left side of the body are involved. It can affect the large joints and small joints (finger and toe joints) in the body, and also may affects the joints in the neck and jaw.

A blood test marker called Rheumatoid factor may be present in your blood or it may be absent. The persistent presence of the Rheumatoid factor generally tells your doctor that your disease is likely to be more aggressive and severe, and will need stronger medications from the beginning. If the arthritis begins in teenage years, it may be very similar to adult type rheumatoid arthritis especially if the rheumatoid factor is positive.

Enthesitis related arthritis (ERA)

Usually occurs in older children (e.g. age 8 and above) and it is usually only a few joints (less than 4) are affected. The joints commonly involved are the knees, hips and lower back (sacro-iliac joint) .This type is more likely to affect boys and may go on to develop stiffness in your neck and back in your teenage years or as a young adult. You may also have ‘enthesitis’ which is inflammation that affects the parts where your tendons, ligaments or joint capsule attaches to bone. Examples of enthesitis are plantar fasciitis (pain in your heel) or Archilles tendinitis (pain at the back of your heel where your Archilles tendon attaches to the calcaneum). Some may have family members with similar back problems (anklylosing spondylitis). Uveitis is also common and this type tends to cause an acute painful eye.

Psoriatic arthritis

Psoriasis is a skin disease where there is a scaly red rash which usually occurs in the scalp or near joints which can run in families. Sometimes you can also get arthritis if you have this skin disease. The problem in children is that the arthritis may appear before the skin rash appears and therefore the diagnosis is sometimes not made till later. Your doctor will need to check your finger and toenails carefully to look for early signs of the disease and also various parts of your body like your scalp especially to look for the skin lesions. The doctor will also ask if any of your immediate family members suffer from psoriasis.

Systemic JIA

This is a rare type of JIA. It can affect children and young people of all ages. Boys are affected as common as girls. In this form of arthritis, joint pain is part of a systemic illness. You may have a high spiking fever (usually one or two spikes a day that may come and go quickly) and also a rash that comes and goes which is likely to be present when you have fever. You will also feel ill especially when you are febrile and have loss of appetite, loss of weight, and may have enlarged lymph nodes (glands in your neck, under your armpit and even your groin). Your spleen and liver may be enlarged and it can also affect your heart (pericarditis) and lungs (pleuritis).

Sometimes in the first few weeks of the illness, the joints may not be swollen yet, and you may just have fever and the rest of the symptoms. The diagnosis may be uncertain at this point in time and lots of test will need to be carried out to confirm the diagnosis. This process can be worrying but is absolutely necessary to ensure that we are not missing out on other diseases which can give a similar picture and are potentially life-threatening like acute leukaemia.

What effects can JIA have on your body ?

Many children and young people with arthritis feel well, are fully active and have normal growth and puberty. Sometimes though, having arthritis can cause problems with everyday activities like walking, bending, getting dressed or bathing, and can have an effect on your general health and development.

Varying Symptoms

Symptoms of arthritis can vary from day to day. Inflamed joints (when arthritis is ‘active’) can be warm to touch, may look red and swollen or be painful to move. Typically you would feel stiffer in the morning and after periods of resting. Flare-ups (when arthritis gets worse) can happen after viral infections, stress, changes in medication or sometimes for no apparent reason. Sometimes the arthritis stops being active but they may still be painful and limit your ability to do things.

Eye inflammation

Uveitis is inflammation in the eye and this can happen with JIA. If uveitis occurs, it can cause blurring or even progressive loss of vision leading to blindness. It does not always cause pain or redness in the eye and may only be picked up with frequent checks by the Ophthalmologist (eye doctor), who will check your eyes with a slit lamp. Therefore it is important to have your eyes checked at regular intervals as recommended by the Ophthalmologist even if you have no problems with your eyes.

General health

Having arthritis can affect your general health. Flare-ups can reduce your appetite, may cause anemia, and make you feel ‘run down’ and tired. You should take care to clean your teeth regularly and properly to prevent tooth decay and gum disease.

Osteoporosis

Osteoporosis (thinning of the bones) usually causes problems in older people. However children and young people with arthritis have a higher risk of developing osteoporosis. This is partly due to the effect of the body’s inflammation on the bones, the medications that you sometimes take as well as the lack of mobility and exercise especially when the arthritis is active. We all know that diet, exercise and general health when you are young are very important in strengthening your bones. Therefore, apart from trying to control your arthritis, it is very important to take steps when you are young to help prevent osteoporosis. You should try to exercise daily (walking is great) and also eat foods that have a high calcium content like dairy products (for example, milk, cheese, yoghurt)

Growth problems

Sometimes if your arthritis is not controlled, the bones around the inflamed joints can either grow faster (overgrowth) or slower (undergrowth). Usually if you have arthritis of a knee, the affected leg can grow faster causing you to have legs which are different in length. However, if you have arthritis of the wrist, feet or jaw joint, your hand or feet might be smaller and your jaw might also be smaller due to undergrowth of those bones. The problem with the jaw can sometimes lead to overcrowding of your teeth and your dentist will be able to advise you accordingly.

At other times, children or young people with more generalized or severe disease may have overall poor growth leading to them being smaller and shorter. This is especially for children or young people with systemic onset disease or Polyarthritis. In addition, their puberty may be delayed , sometimes up to several years but on the whole, they will achieve puberty.

How is JIA treated ?

The aim of treatment is to:
• Control the symptoms of arthritis (pain, swelling and stiffness)
• To enable you to lead an active life at school or college
• To enable you to enjoy an active family and social life
• To enable you to do as well as you can at school
• To help you become an independent adult
• To minimize any possibility of long term damage to your joints

There is a great deal of on-going research in the treatment of arthritis but at the moment, there is no cure. You can help yourself in the following areas:

• Medicines
• Physiotherapy and Occupational Therapy
• General Health and healthy lifestyle
• Joint Surgery
• ‘Natural’ and ‘complementary’ treatments

1) Medicines

Medicines cannot cure arthritis but they can control the symptoms and help reduce the possibility of joint damage. Some of the medicines are as follows:

Non steroidal anti-inflammatory drugs (NSAIDs)

These often help to reduce pain (sometimes hence known as painkillers), stiffness and swelling of arthritis. There are many different NSAIDs like naproxen, ibuprofen, diclofenac, meloxicam and piroxicam. To be effective, they have to be taken regularly and not only when you have pain in your joints. Side effects include indigestion and this can be lessened by taking them with food. If you get indigestion, you should report this to your doctor. Some rare side effects include mood disturbance and very rarely, kidney damage. If you have asthma, please inform your doctor. If you are taking NSAIDs on a long term basis, your doctor will keep and eye to monitor for the side effects but checking your blood or urine.

Corticosteroids (Prednisolone)

These control inflammation and hence the pain, stiffness and swelling of arthritis very well. They work quickly over a few days and the effects can be seen. Short courses (lasting for a few days) or low doses tend to have no side effects.

Some side effects, which can occur with large doses, can include an increase in appetite and weight gain, facial changes where the face appears to swell up and increase in downy hair sometimes especially over the back. Long term usage can lead to slowing of growth, high blood pressure, high blood sugar, easy bruising and osteoporosis. Sometimes it can cause cataract and also high pressure inside the eye (glaucoma).

Steroids can also increase the risk of infection. Chicken pox can be very serious if you have not had it before and you catch it when you are on steroids. You need to inform your doctor the moment you come into contact with someone with chicken pox and you will need a special medicine (Acylovir) the moment you start developing any chicken pox lesions.

You should also NOT receive any live vaccines like BCG, oral polio, MMR, rubella and chicken pox vaccine. Ask your doctor if you intend to have any of these. However, you can have vaccines like Hepatitis B, Diptheria or tetanus toxoid.

Your doctor will aim to limit these side effects of steroids by prescribing them in as low a dose as possible and for the shortest duration of time. However sometimes due to the severity of disease, you may need to be on continuous steroids for long term but your doctor will monitor you carefully for these side effects.

Steroids can be given by:
• Tablets
• Injections into muscle
• Injections into the joint (are very effective for many months, are safe and have very few side effects)
• A drip into a vein

You must never stop steroids suddenly as steroids can stop the normal production of cortisone from the adrenal glands and you can become very ill. You need to follow the instructions given by your doctor in reducing or stopping the steroid dosages, and if you are ever about to run out of steroid pills, please inform your doctor,

Disease modifying anti-rheumatic drugs (DMARDs)

These drugs can dampen down the inflammation and are important as they can reduce joint damage caused by the arthritis. Also, by helping to control the arthritis, it may enable your doctor to reduce the amount of steroids that is needed to keep you well.

The most common drugs used in JIA are Methotrexate and to a lesser extent Sulphasalazine. These drugs appear to be effective with many patients going into remission (no active arthritis) and feeling very well. However, these drugs are slow to become effective and you may not notice any benefit for several months. These drugs are considered immunosuppressives and they act by suppressing the immune system that normally defends the body against infection and disease and as such like steroids, you are at a slightly higher risk for infections. If you have chicken pox, you will need to also contact your doctor immediately.

Methotrexate is a common drug which is used in children with arthritis. It is normally taken only once a week either by mouth or with an injection into the fat under the skin (called a subcutaneous injection). It must be taken on the same time at the same day every week. When given by mouth, Methotrexate must be eaten on an empty stomach first thing on waking up, at least ½ an hour before breakfast. At higher dosages, sometimes the preferred route is by an injection. The commonest side effect is feeling queasy or nauseous after taking Methotrexate for a few hours. Other minor side effects include mouth ulcers and diarrhea. Some rarer more serious side effects include effects on the liver (elevation of the liver enzymes), bone marrow (suppression of the bone marrow causing low cell counts) and lung problems. These side effects can be detected easily and if detected early are reversible when the drug is stopped. Your doctor will need to do blood tests on you at regular intervals to look out for these side effects.

Sulphasalazine is another DMARD. It also takes a few months to be effective. Some side effects include headaches, loss of appetite and skin rashes. It can also sometimes cause low blood counts and liver problems. You will also need regular blood test to be done to assess the impact of the drug.

Other DMARDs that may sometimes be used include Hydroxychloroquine or Leflunomide. Gold salts and Penicillamine are generally no longer used in children. Some children may require other forms of therapy like Intravenous Immunoglobulin infusions.

Biologics

Over the last decade, research has revealed a new group of drugs called biologics which are now available for the treatment of arthritis. Biologics have offered hope for some of those whose arthritis is not controlled on conventional DMARDs. These drugs also modulate the immune system and aims to control the inflammation. These include anti-TNF (tumour necrosis factor) therapy like Etanercept, Infliximab and Adalimumab. Etanercept and Adalimumab are given by injections into the fat under the skin (subcutaneous injection) whilst Infliximab is injected into the vein at scheduled intervals. Other biologics include anti-IL 1 (Anakinra) and anti-IL 6 (Tocilizumab). Various other drugs are also still being developed.

2) Physiotherapy and Occupational Therapy

Physiotherapy and Occupational Therapy are just as important as your medicines to help your arthritis. These can result in pain relief, improve your ability to get on with life, and exercises are useful to stretch soft tissues, build up muscles and improve your heart and lung fitness.

Exercise

As a general rule, you should try and exercise everyday, and this includes general and specific exercises. Try to set aside 15-20 minutes each day to ‘work out’. This need not be energetic but you should check that each joint in your neck, spine, arms and legs move freely.

Exercises are important because :
1) they maintain a better range of movement and mobility
2) they improve muscle stamina, strength and suppleness
3) they improve your general fitness especially regular exercises like swimming.
4) they improve your sleep pattern
5) they will build up bone density and strength which is especially important while the skeleton is developing and will counteract osteoporosis. Weight bearing exercises (such as walking) will help prevent osteoporosis.

Splints

Splints are devices which are made from thermoplastic material that are moulded around your arms and legs to keep the body part in correct position. Splints may be used to rest or protect a joint. It can help ease the pain in your joints and using it all the time can help prevent joint deformities. It is important for you to wear your splints as advised.

3) A Healthy Lifestyle

Everyone can improve their general health through healthy eating, regular exercise, adequate sleep and avoiding smoking, illegal drugs and alcohol.

Regular exercise will improve your fitness level (heart and lungs), protects against osteoporosis and also helps you to relax. For example riding a bicycle or swimming is a good way to keep mobile and to keep you fit.

You should aim for a well-balanced diet with plenty of fruit and vegetables, and avoiding too much fat. You should also have lots of calcium rich foods (e.g. Milk, cheese, and yoghurt) to prevent osteoporosis. There is no specific food that needs to be avoided unlike in an adult type of arthritis called ‘gout’ whereby patients need to reduce foods that can cause a rise in their body’s uric acid. There are lots of tales about diet and arthritis but these are often unproven. As you are growing, it is very important that you get a well balanced diet to enable optimal growth for your body.

4) What about ‘natural’ or ‘complementary’ therapies?

There are many people who make claims for certain treatment that are not ‘conventional’ medicines. Some may be helpful but there is little evidence to supports their use in everyone with JIA. The internet has a lot of information on many products. Be cautious as many products are advertised heavily and can be very expensive and may not work. Discuss this with your doctor.

5) Joint surgery

Occasionally JIA can cause joint damage that causes pain and difficulties with daily activities. Joint replacements are now very successful treatment in adults with arthritis. They are effective in relieving pain and improving mobility and can last for many years. Joint surgeries are only done when a person has stopped growing.

Why do I need to be seen regularly ?

You will be seen regularly so that your doctor can find out how you are doing and also to examine your joints. They may want to do some test to make sure that the medicines are working for you and to monitor for side effects with the medicines. Your doctor will also want to check your growth (height and weight). The visit to the clinic will be an opportunity for you to ask any questions that you have regarding your disease as well as any concerns with school or other problems.

What happens to you in the future ?

Once you are diagnosed with arthritis, unfortunately no doctor can predict how your arthritis will develop. There is no test that will tell you how the disease will affect you individually or how long it will last. In most cases, children’s arthritis has a good outcome, with 50% – 70% of people having no problems when they are adults. 

In about 30%- 50% of cases, arthritis can remain active into adult life. Some young adults with JIA have joint damage that limits their daily activities to some extent and a few may need joint replacements. Some may be physically smaller than average or have osteoporosis as a result of their arthritis and /or treatment with steroids.

A successful outcome in JIA requires many things. How you approach it can make a big difference and it is important that you remain positive. You need to be looked after by a team experienced in dealing with arthritis. You can help by doing your exercises, wearing your splints as suggested and taking your medication regularly. Many young people with JIA have achieved highly, with good jobs and have satisfying fulfilling lives, and you can be one of them !

 *** this info is taken from Arthritis Foundation of Malaysia website***