Home Arthritis in Children
From the Arthritis Foundation of America

Introduction

As parents of a child with arthritis, much of the responsibility for the health care and emotional well-being of your child with arthritis will depend on you. You will be the ones to:

-be sure she sees all members of her health-care team as needed;
-see that she takes her medications are prescribed;
-watch for any side effects the drugs may cause;
-help her do exercises to relieve pain and stiffness;
-provide encouragement and support to her and other family members; and
-work with teachers and school officials to make sure her needs are met.

For this reason, it is very important for you to know as much as possible about your child’s arthritis and its care, as well as the effects it can have on family life. This booklet provides a starting point for discussions with your child’s health-care team.

What Kinds of Arthritis Affect Children?

The word arthritis refers to inflammation (that is, swelling, heat and pain) involving the joints. Arthritis is frequently a chronic illness, meaning that it may last for months or years. About 200,000 children - or 2.9 in every 1,000 - in the United States have some form of arthritis.

The most common form of arthritis in children is juvenile rheumatoid arthritis (JRA). However, children may also be affected by arthritis as a feature of other diseases including those listed on the following pages.

-Systemic Lupus Erythematosus, a chronic inflammatory disease characterized by fever and rash that may attack organs such as joints, kidneys, the brain, lungs and heart.

-Juvenile dermatomyositis, a disease that causes a skin rash and weak muscles in children, and may be accompanied by swollen joints.

-The spondyloarthropathies of childhood, diseases in children that involve the spine. In some but not all children with these diseases, a protein called HLA-B27 is found on the white blood cells. The spondyloarthropathies of childhood include:

-Ankylosing spondylitis, a type of arthritis which primarily affects the spine and hips. It usually occurs in males.
-Reiter’s disease, a form of arthritis that may cause inflammation of the urinary tract and eyelids, mouth ulcers and/or skin rash.
-B-27 arthritis, a form of arthritis that occurs more often in older boys and affects only a few joints - usually the back, and large joints of the legs such as hips, knees and ankles. If particular changes are seen in X-rays, B-27 arthritis may be reclassified as ankylosing spondylitis.
-Psoriatic arthritis, a type of arthritis that may occur with the skin condition, psoriasis. It affects both boys and girls.
-Scleroderma, a disease that can affect the skin, joints, blood vessels and internal organs.
-Inflammatory bowel (Crohn’s) disease, a disease that can affect the intestines, causing diarrhea and abdominal pain. It can be associated with arthritis and fever; these sometimes appear before the digestive symptoms.

What is Juvenile Rheumatoid Arthritis (JRA)?

Juvenile rheumatoid arthritis (JRA) affects about 71,000 children in the United States. It is a disease of the joints that may also affect other organs. JRA is often a mild condition which causes few problems, but in severe cases it can produce serious complications. Its signs and symptoms may change from day to day, even from morning to afternoon. Joint stiffness and pain may be mild one day but become so severe the next that the child cannot move without great difficulty.

Periods when the arthritis is particularly active are called flares.

There are at least three forms of JRA. Each form begins in a different way and has different signs and symptoms. The three forms are:

-Polyarticular JRA, which affects five or more joints;
-Paucuarticular JRA, which affects four or fewer joints; and
-Systemic JRA, which affects both the joints and the internal organs.

Each of these forms is discussed in this booklet.

What Causes JRA?

No one knows the cause of JRA, but we know that it involves abnormalities of the immune system. The immune system defends our bodies against bacteria, viruses and other foreign substances.

Some research suggests that in autoimmune diseases such as JRA, one type of white blood cells called lymphocytes loses the ability to tell parts of ones body, such as cartilage, from harmful agents like bacteria or viruses. This results in the release of chemicals that can damage the body’s own tissues, in a process called inflammation.

The painful joint swelling children with JRA experience is one example of inflammation; another is iridocyclitis, an inflammation in the front of the eye, near the iris.

There is a great deal of research to find out what causes arthritis. Once this in known, it may be possible to cure this illness or prevent it from starting.

We do know that JRA is not contagious, so your child didn’t catch it from anyone and can’t give it to anyone. We also know that heredity plays some part in the development of several forms of arthritis. However, the inherited trait alone does not cause the illness. We think that this trait along with some other unknown factors triggers the disease. It is unusual for more than one child in the family to have arthritis.

Common Features of JRA

Arthritis is characterized by four major observable changes in the joint. However, since JRA affects each child differently, your child may not experience all of these changes. Children also vary in the degree to which they are affected by any particular symptom. The most common features of JRA are joint inflammation, joint contracture, joint damage and altered growth. Other symptoms your child may experience include joint stiffness following decreased activity, and muscle and other soft-tissue weakness.

Joint Inflammation

Joint inflammation is the most common symptom of JRA. It causes heat, pain, swelling and stiffness in joints. The lining of the joint, called the synovium, becomes swollen and overgrown and produces too much fluid. This causes swelling, stiffness, pain, warmth and sometimes redness of the skin over the affected joints.

Joint Contracture

Since it usually hurts to move an inflamed joint, your child may hold it still in a bent position. If she holds a sore joint in a fixed position for a long time, the muscles around the joint will become stiff and weak.

After a while, the tendons may tighten up and shorten, causing a deformity called a joint contracture. Doctors usually prescribe an exercise program to prevent this from happening.

Joint Damage

In some children with severe disease, long-lasting inflammation damages the joint surfaces. This is called joint erosion, and can cause pain and limitation of motion.

Altered Growth

Sometimes joint inflammation either speeds up or slows down the growth centers in bones. This can make the affected bones longer, shorter or bigger than normal. If the growth centers in many bones have been damaged by inflammation, a child may stop growing entirely. If no damage has occurred, however, the child will usually continue to grow once the JRA is under control.

Features of Specific Types of JRA

Each of the three types of JRA has specific features. The most common features for each are discussed below, along with other possible features.

Polyarticular JRA

Polyarticular means “many joints”. In this form of JRA, five or more joints are affected. Girls get polyarticular arthritis more often than boys. Because it can be severe, the most powerful medications are recommended for this type of JRA.

Most Common Features
-usually affects the small joints of the fingers and hands;
-can also affect weight-bearing and other joints, especially the knees, as well as the hips, ankles and feet, neck and jaw; and
-often affects the same joint on both sides of the body.

Other Possible Features
-low fever;
-a positive blood test for rheumatoid factor; and
-rheumatoid nodules, or lumps, on an elbow or other point of the body that receives a lot of pressure from chairs, shoes or other objects.

Pauciarticular JRA

Pauciarticular means “few joints”. In this form of JRA, four or fewer joints are affected.

Most Common Features
-usually affects the large joints (knees, ankles or elbows);
-often affects a particular joint on only one side of the body; and
-may cause iridocyclitis, and eye inflammation

Systemic JRA

Systemic means “affecting the body generally”. Systemic JRA affects a child’s internal organs as well as the joints, and may take months to diagnose. This is the least common form of JRA. Boys and girls are equally likely to get this kind of JRA. In some, the systemic symptoms of the disease and the fever may go away completely, although the joint-related symptoms of arthritis may remain.

Most Common Features

High fevers usually start in the late afternoon or evening. The child’s temperature may go up to 103 degrees or higher and then return to normal within a few hours. Chills and shaking often go along with the fever and the child may feel very sick. Periods of fever can last for weeks or even months, but rarely go on for more than six months.

Pale red spots often appear on the child’s chest and thighs and sometimes on other parts of the body. This rash comes and goes for many days in a row.

Joint inflammation may accompany the fever or may not start until weeks or even months later. Some children have severe pain in their joints when they have a fever and then feel much better when their temperature goes down. Joint problems can be a major long-term difficulty for children with this kind of arthritis.

Other Possible Features
-inflammation of the outer lining of the heart, the heart itself or the lungs;
-anemia (low red blood count);
-a high white blood cell count in the blood; and
-enlarged lymph nodes, liver and spleen.

Regular visits to your child’s doctor are important so these problems can be checked and treated from the beginning.

How is JRA Diagnosed?

In order to make a diagnosis of JRA, the arthritis must have been constantly present for six or more consecutive weeks.

The signs and symptoms of JRA vary from child to child. There is no single test that makes the diagnosis of JRA. Therefore, your doctor may go through many steps to find out if your child really does have JRA. The main steps involved in diagnosis are discussed below.

Your Child’s Health History

To make a correct diagnosis, the doctor will ask questions about your child’s recent symptoms, medications she is taking, and any previous medical problems.

The doctor may also wish to rule out other possible causes of arthritis through various tests. Many viral infections can lead to temporary joint problems in children, but in these cases the arthritis usually goes away rapidly. Other diseases can cause arthritis. Sometimes a bacterial infection of bone or cartilage can cause joint swelling or pain. Prompt diagnosis is important to allow proper antibiotic treatment.

The doctor may also want to know if other members of the family have had any other form of arthritis, since some forms may be inherited.

Physical Examination

During the physical examination, the doctor will look for joint inflammation, rashes, nodules and eye problems.

The doctor must be able to find evidence of joint inflammation to be sure the problem is JRA. A child who complains of aches and pains, but shows no joint changes, may not have JRA. In a few cases, a physically healthy child experiencing acute emotional stress may complain of sore joints.

An ophthalmologist may also need to examine your child’s eyes to check for signs of iridocyclitis.

Laboratory Tests

Although there are several laboratory tests that may support a diagnosis of JRA, there is no single test that provides positive proof one way or another. The most common tests are:
-erythrocyte sedimentation rate (sed rate);
-rheumatoid antibody test (ANA);
-HLA-B27 typing;
-hemoglobin test; and
-urinalysis.

If the diagnosis is particularly hard to make, the doctor may do additional tests to rule out other diseases.

X-ray Examinations

X-ray examinations of joints may be helpful early in the course of the illness to find out if another condition such as a bone infection, tumor or fracture is causing the problem. Later on, X-rays may be used to check on joint damage, or changes in bones. X-rays of the spine help the doctor tell if ankylosing spondylitis is present.

Your child’s physician may also suggest magnetic resonance imaging (MRI), a technology that uses magnetic waves to provide images of the inside of the body without harmful radiation.

Joint Fluid and Tissue Tests

A sample of fluid from one or more joints may be withdrawn by a needle and examined to find out if there is an infection in the joint.

Sometimes the doctor will take a small bit of tissue from a joint or a nodule for examination in the laboratory. This is called a biopsy.

Does JRA Have Long-Term Effects?

JRA is a chronic disease - one that may last for many years. Eventually, there are good chances that your child will get well and experience no serious, permanent disability. Children with JRA can usually keep up with school and many social activities. Some changes may need to be made when the child has a flare or if there has been joint damage.

Sometimes, the signs and symptoms of JRA may go away. When this happens, it is called a remission. A remission may last for months, or years, or even forever. But no one can be sure this will happen in your child.

While most children with JRA do well in the long run, parents should be aware of possible long-term consequences. Children with pauciarticular JRA have a higher risk of chronic eye inflammation. Some children with pauciarticular or systemic JRA may have serious joint problems or develop other long-term complications, such as decreased growth.

There is no fast and simple solution to JRA. The most important thing you can do is work with your doctor and other health professionals to manage the disease and keep it under control.

How JRA is Treated

Your child’s treatment program will be based on the kind of arthritis she has and on her specific symptoms. The goals of any treatment program for juvenile rheumatoid arthritis are to:
-control inflammation;
-relieve pain;
-prevent or control joint damage; and
-maximize functional abilities.

Your child’s treatment program will usually include medications, exercise, eye care, dental care and healthy eating practices. Other treatments, such as surgery, may be necessary for special long-term problems.

Some physicians have also found that pain can be lessened by combining medical treatment with techniques such as progressive muscle relaxation, meditative breathing and guided imagery.

Because so many techniques are used to treat children with JRA, the ideal type of care is sometimes called team care or coordinated care. Your child’s health care team will include many different specialists who work together to offer your child a complete treatment program. Pediatric rheumatology centers found in many major medical centers offer this care in one location. If you do not live near a pediatric rheumatology center, your child’s physician will refer you to the specialists she needs.

Note: Because there is no cure for JRA, you may be tempted to try an unproven remedy. Some unproven remedies are harmless, but others are dangerous. Bring any questions to your doctor.

Medications

Several kinds of medications are used to control inflammation and relieve the pain of arthritis. Your child’s doctor will generally try nonsteroidal anti-inflammatory drugs (NSAIDs) or aspirin before adding, if necessary, a more potent anti-rheumatic drug or corticosteroid.

All drugs may have side effects, but the effects vary from child to child. One child may respond well to one drug, while another with a similar condition may not benefit or may suffer side effects from the same medication. It is very important that your child’s urine, blood and liver function are tested at regular intervals to make sure everything is normal.

Because many drugs take weeks or months to show any benefit, the physician may keep your child on a particular drug for some time before trying another, unless there are serious side effects.

An important message about arthritis medications: Don’t adjust or stop them without first asking your child’s doctor.

Nonsteroidal Anti-Inflammatory Drugs (NSAIDs)

Your child’s physician will probably prescribe an NSAID as a first step to control swelling and pain. NSAIDs are helpful in all types of JRA. They work by limiting the release of irritating chemicals by the white blood cells. NSAIDs can also be effective in lowering fever.

NSAIDs commonly used to treat JRA include ibuprofen, naproxen and tolmetin. Most NSAIDs are equally effective, but a particular child may respond better to one than to another. NSAIDs are available by prescription or over the counter.

Dose: These drugs may be given as a pill or liquid. The dose varies, depending on the specific NSAID used.

Possible side effects: NSAIDs can have side effects including:
-stomach pain, nausea and vomiting;
-anemia;
-headache;
-blood in the urine;
-severe abdominal pain and peptic ulcer;
-fragility and scarring of the skin (especially with naproxen); and
-difficulty concentrating in school, in some cases.

Aspirin

Aspirin may be used to control swelling and pain in joints and reduce fever in children with systemic JRA.

Dose: Children with JRA for whom aspirin is prescribed must take large amounts of it three or four times a day. Young children should not suck or chew on the aspirin because this may erode the chewing surfaces of the teeth and irritate the gums. Instead, try pre-crushing the dose and having the child swallow it in a small amount of a favorite food such as applesauce or yogurt.

Possible Side Effects: Aspirin may cause:
-stomach pains or stomach bleeding (giving aspirin with food or and antacid may help);
-toxic reactions. Both you and your child should be aware of these signs:
-rapid or deep breathing;
-ringing in the ears;
-decrease in hearing;
-drowsiness;
-nausea;
-vomiting;
-irritability;
-unusual behavior; and
-black tarry stools

-Reye’s Syndrome, a rare disease which sometimes occurs in children who have the chicken pox or the flu, and who are also taking aspirin. The symptoms of Reye’s syndrome are:
-frequent vomiting;
-very painful headaches;
-unusual behavior;
-extreme tiredness; and
-disorientation.

If your child is taking aspirin and develops chicken pox or flu, she should stop taking the aspirin for a while. Your doctor will tell you what to do if this happens.

More Potent Anti-Rheumatic Drugs

The more powerful anti-rheumatic drugs are not usually given by themselves; they are most often effective when given in addition to an NSAID. They are usually prescribed when NSAIDs alone have not been effective or when joint damage occurs, usually in children with polyarticular disease. These more powerful drugs are often able to limit the amount of inflammation seen in severe JRA.

Methotrexate

Methotrexate works primarily by decreasing excessive white blood cell activity. Its use in children is fairly recent. However, methotrexate has been found to be effective in treating children with active polyarticular JRA and some with severe pauciarticular disease.

Dose: Methotrexate is normally given weekly in low doses, usually as a pill taken by mouth. It may also be given by injection.

Possible side effects: In the low doses at which methotrexate is usually prescribed, few serious side effects have been reported. However, regular laboratory monitoring is important. Possible side effects may include:
-nausea;
-mouth sore;
-diarrhea;
-low white blood cell count;
-lung irritation;
-sinus infection;
-liver irritation. Anyone taking methotrexate, including teenagers, should avoid all alcohol intake to lower the risk of irritation the liver; and
-risk of birth defects if taken during pregnancy.

Gold

The gold used in gold treatment is actually a liquid gold salt. It works by interfering with several different functions of white blood cells.

Gold treatment is used to ease morning stiffness and control swelling and pain in joints.

Dose: Gold is given in two ways - by injection in to the muscle, or as oral gold in a capsule taken by mouth. Injections are usually given every week for five or six months, then once or twice a month for as long as necessary. Oral gold (auranofin) is taken daily.

Four to six months may pass before a child responds to gold treatment. Gold is not effective in all children; when it is, treatment may need to be continued for many years.

Possible side effects: Gold can have side effects which may make it necessary to stop treatment. Regular laboratory tests are needed to detect and adverse reactions. Side effects may include:
-skin rash;
-mouth sores;
-kidney problems;
-a low blood count; and
-anemia.

Hydroxychloroquine (Plaquenil)

Hydroxychloroquine (hi-DROCKS-ee-CLOR-o-kwine), sold under the trade name Plaquenil (PLA-kwe-nill), is another drug found to work in some children with polyarticular JRA and other forms of arthritis. While not helpful in all cases, Plaquenil may be useful, particulary when methotrexate or gold have not been completely effective.

Dose: Plaquenil is given in pill form to control swelling and pain in joints. Your doctor will determine the correct amount.

Possible side effects: side effects of Plaquenil may include:
-upset stomach
-skin rash; and
-eye damage. A child taking the drug should be checked every six to 12 months by an ophthalmologist.

Keep this drug out of reach of small children. An overdose of this medication can be fatal.

Corticosteroids

Corticosteroid (CORE-ti-koh-STARE-oyd) drugs are used to treat JRA, especially the systemic form, when it is very severe and has not responded to other drugs.

Corticosteroids used to treat JRA include prednisone and cortisone. These drugs contain cortisone and are not related to the synthetic male-hormone steroids some athletes use.

Corticosteroids are used to control swelling and pain in joints; control pericarditis, pleuritis, continuous high fever or severe anemia; and control iridocyclitis (when given as an ointment or eye drops).

Corticosteroids work swiftly and effectively. However, because of their side effects, they are used with caution.

Dose: If corticosteroid drugs are prescribed, the lowest possible dose will be used for the shortest length of time. Usually, the drug is taken by mouth as a pill or liquid, or it can be given intravenously directly into a vein. It can also be given as an injection into the joint itself, or into a muscle or vein. Other types of steroids include eye drops used to treat iridocyclitis and steroid creams for skin problems.

Possible side effects: Corticosteroids taken as a pill or intravenously for long peroids of time may cause severe problems such as:
-high blood pressure;
-osteoporosis (softening of the bones);
-slowing of the child’s growth rate;
-reduced resistance to infection;
-sudden mood swings;
-increased appetite and weight gain; and
-increased risk for ulcers.

Medicines for the Future

Biologicals are a new class of medications made of synthetic proteins. They are still in the research phase. It is believed that biologicals may slow or stop the harmful immune response that leads to inflammation in the joint.

Biologicals now being studied include monoclonal antibodies, receptor inhibitors, peptide vaccines and intravenous gammaglobulin.

Exercise

Exercise is a very important part of the treatment for JRA. For children with arthritis, the purpose of regular exercise is to:
-keep joints mobile;
-keep muscles strong;
-regain lost motion or strength;
-make everyday activities like walking or dressing easier; and
-improve general fitness and endurance.

Kind of Exercise

There are two kinds of exercise your child might do: therapeutic exercise and sports and recreational activities.

Therapeutic Exercise

While medications reduce pain and inflammation, only therapeutic exercise can restore lost motion in a joint. Therapeutic exercises make it easier for your child to walk and perform other activities of daily living, like opening jars and writing. Range-of-motion exercises keep joints flexible and are especially important for children who have lost motion in a joint, or whose joints have become fixed in a bent position. Strengthening exercises build muscles.

A physical or occupational therapist will show your child how to perform therapeutic exercises at home. Most exercises must be done every day. Hot baths, hot packs and/or cold treatments before exercise can make the therapy easier. The therapist can show you how to make the exercises part of play activities if your child is very young.

Sports and Recreational Activity