Juvenile Rheumatoid Arthritis

Rheumatoid arthritis, often called RA, is a chronic (long-standing) disease that damages and eventually destroys the joints of the body. The damage is caused by inflammation, a normal response of the body’s immune system. Arthritis means “joint inflammation.”

• The inflammation in the joints causes pain, stiffness, and swelling as well as many other symptoms.
  
  
• The inflammation often affects other organs and systems of the body as well.

• If the inflammation is not slowed down or stopped, it eventually destroys the affected joints and other tissues.
  
  

Juvenile rheumatoid arthritis (JRA), or juvenile arthritis, is not a single disease, but a group of diseases. What they all have in common is chronic joint inflammation. Besides this common feature, these diseases are very different in their symptoms, their treatments, and their outcomes.

• Pauciarticular disease affects only a few joints, fewer than 5. The large joints, such as the shoulder, elbow, hip, and knee, are most likely to be affected. This type of JRA is most common in children younger than 8 years. Children who develop this disease have a 20-30% chance of developing inflammatory eye problems and need frequent eye examinations. Children who develop this disease when older than 8 years have a higher-than-normal risk of developing an adult form of arthritis. About 50% of all children with JRA have this type.
  
  
• Polyarticular disease affects 5 joints or more, sometimes many more. The small joints such as those in the hands and feet are most likely to be affected. This type can begin at any age. In some cases, the disease is identical to adult-type RA. This type accounts for about 30% of cases of JRA.
  
  
• Systemic disease affects many systems of the body. Children may have high fevers, skin rashes, and problems caused by inflammation of the internal organs such as the heart, spleen, liver, and other parts of the digestive tract. It usually, but not always, begins in early childhood. Medical professionals sometimes call this Still’s disease. This type accounts for about 20% of cases of JRA.
  
  

Children with JRA may experience complications specific to their type of JRA.

• The most common complications in children with JRA relate to adverse effects of medications taken to treat the disease, particularly nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin). When taken frequently, these drugs can cause irritation, pain, and bleeding in the stomach and upper intestine. They also can cause problems in the liver and kidneys that often have no symptoms until they are very severe. In some cases, the child must undergo frequent blood tests to screen for these problems. 
  
  
• Some children with JRA have emotional or psychological problems. Bouts of depression and problems functioning in school are the most common. 
  
  
• The death rate in children with JRA is somewhat higher than in healthy children. The highest death rate in children with JRA occurs among patients with systemic JRA who develop systemic symptoms (eg, pleural and pericardial disease). JRA can also evolve into other diseases, such as systemic lupus erythematosus (SLE) or scleroderma, which have higher death rates than pauciarticular or polyarticular JRA. 
  
  

Treatment for JRA, like that for adult-type rheumatoid arthritis, has improved dramatically in the last 30 years, thanks mainly to the development of new medications.

The cause of juvenile rheumatoid arthritis is unknown. Like adult-type RA and many other related diseases, JRA is an autoimmune disease. This means that the body’s immune system mistakenly attacks the tissues it is supposed to protect. 

• The chronic inflammation of the synovium (the lining of the joints) is linked to greater-than-normal activity of the immune system.
  
  
• Normally, the immune system fights “assaults” by “invaders” such as infections or blood or tissue from another person.
  
  
• The immune system produces specialized cells and proteins, which are released into the bloodstream to fight off the “invaders.” One important type of immune protein is called an antibody.
  
  
• In autoimmune diseases, the cells and antibodies of the immune system attack the body’s own tissues. In the case of arthritis, the attack is directed against the synovium, which becomes inflamed.  
  
  
• The inflammation causes the synovium to thicken and grow abnormally. As the synovium expands outside of the joint, it presses on and eventually damages the bone and cartilage of the joint and the surrounding tissues such as ligaments and tendons.
  
  
•  We do not know what causes the inappropriate autoimmune response.

The symptoms of juvenile rheumatoid arthritis vary greatly from child to child. They may be very mild, very severe, or anything in between, and they may change over time, sometimes overnight. Fluctuations, in which the symptoms get worse (flare) and then get better or go away completely (remission), are fairly typical of JRA.

• Joint pain, stiffness, and swelling: These are the most common symptoms of JRA, but many children do not recognize, or do not report, pain. Stiffness and swelling are likely to be more severe in the morning.
  
  
• Loss of joint function: Pain, swelling, and stiffness may impair joint function and reduce range of motion. Some children are able to compensate in other ways and display little, if any, disability. Severe limitations in motion lead to weakness and decreased physical function.
  
  
• Limp: A limp may indicate a particularly severe case of JRA, although it also may be due to other problems that have nothing to do with arthritis, such as an injury. In JRA, a limp often signals knee involvement.
  
  
• Joint deformity: The joints may grow in an abnormal, asymmetrical way, causing deformities of the extremity involved.
  
  
• Eye irritation, pain, and redness: These symptoms are signs of eye inflammation. The eyes may be sensitive to light. In many cases, however, eye inflammation has no symptoms. If the inflammation is very severe and not reversed, it can cause loss of vision. The most common types of eye inflammation in JRA are uveitis and iritis. The names refer to the part of the eye that is inflamed.
  
  
• Recurrent fevers: Fever is high and comes and goes with no apparent cause. Fever may “spike” (go high) as often as several times in one day.
  
  
• Rash: A light rash may come and go without explanation.
  
  
• Myalgia (muscle aches): This is similar to that achy feeling that comes with the flu. It usually affects muscles throughout the whole body, not just one part.
  
  
• Lymph node swelling: This is sometimes called “swollen glands,” but the lymph nodes are not glands. They are small nodules of tissue that work as part of the immune system to help remove certain types of dead cells. Normally, lymph nodes are very small and cannot be felt through the skin. When swollen, they can be felt and often are tender to the touch. Lymph nodes are spread throughout the body, but swollen lymph nodes are noticed most often in the neck and under the jaw, above the collarbone, in the armpits, or in the groin.
  
  
• Weight loss: This is common in children with JRA. It may be due to the child’s simply not feeling like eating. Weight loss with diarrhea suggests possible inflammation of the digestive tract.
  
  
• Growth problems: Children with JRA often grow more slowly than average. Growth may be unusually fast or slow in an affected joint, causing one arm or leg to be longer than the other. General growth abnormalities may be related to having a chronic inflammatory condition such as JRA or to the treatment, especially glucocorticoids (eg, prednisone).

While none of these signs and symptoms point definitely to juvenile rheumatoid arthritis, all warrant a visit to your child’s health care provider.

• Joint pain, swelling, or stiffness that is not due to an injury and lasts more than a few days
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• Loss or limitation of the function of a joint or limb
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• Eye irritation, redness, sensitivity to light, or pain
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• Any loss of vision, even a slight loss
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• Fevers that come and go without explanation
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• Rash that comes and goes without explanation
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• Lymph node swelling with no apparent illness, lasting more than a few days

It is important to remember that many conditions other than juvenile rheumatoid arthritis can cause joint pain, stiffness, and swelling.

• Examples are infections with bacteria or viruses, injury (such as a sprain or fracture), systemic lupus erythematosus, inflammatory bowel disease, Lyme disease, and certain kinds of cancer.
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• Other symptoms of JRA are similarly not specific, meaning that they can be caused by many different conditions. Fever, for example, is a very common symptom of infection.
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• A child who has joint symptoms needs a thorough evaluation by a qualified medical professional. This evaluation will focus on consideration of many conditions and thus arriving at a specific diagnosis.
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• In some children, the symptoms strongly suggest some type of arthritis. In others, the symptoms and signs are more subtle and require careful investigation by the health care provider. Often, a specialist such as a pediatric rheumatologist is consulted to help with the diagnosis.
  
  

The medical interview is a crucial part of making a diagnosis. You will be asked about the following information. It is important that you answer as completely as possible, as this information may help your child.

• Your child’s symptoms and behavior
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• The child’s other medical problems, injuries, and accidents, either recent or in the past
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• His or her vaccinations, medications, and allergies
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• His or her activities, such as sports and games
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• Family medical history (medical problems in the brothers and sisters, mother and father, and their families)
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• The family’s habits and lifestyle
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• The child’s exposures to pets and other animals
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• Recent travel or time spent outdoors, such as camping, hiking, or on a farm
  
  

A detailed physical examination is another critical tool in the evaluation. The examination will include observing, touching, and moving the joints. Muscle strength and flexibility also will be checked. The person conducting the examination looks specifically for evidence of pain, stiffness, swelling, or deformity. The physical examination will cover all systems of the body, with special focus on systems often affected by JRA, such as the eyes, the skin, the heart, and the digestive tract.

Lab tests

There is no lab test that definitely confirms that a child has JRA. The diagnosis is made from a combination of the information gained from the medical interview and physical examination, from a number of different lab tests, and, in some cases, from x-rays and related tests. Because the symptoms must persist at least 6 weeks to be considered JRA, these lab tests may need to be repeated to confirm the diagnosis. After JRA is diagnosed, the tests are done every so often to check disease activity and the success of treatment. All of these are blood tests unless stated otherwise.

• Erythrocyte sedimentation rate (ESR): ESR is a “nonspecific” marker. It does not point specifically to JRA but indicates active inflammation in the body. It is almost always elevated in children with systemic JRA. It usually is elevated in children with polyarticular disease, but is often normal in those with pauciarticular disease.
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• Complete blood cell count (CBC): This test measures the amounts of each type of blood cell in a sample of blood. It also indicates the level of hemoglobin, the protein in blood that carries oxygen around the body. A low level of hemoglobin, called anemia, is common in children with JRA. This test highlights abnormalities in the numbers of various kinds of white blood cells (part of the immune system) or of platelets (which help blood clot). It can be used to distinguish JRA from other conditions that might have similar symptoms. The white blood cell count and platelet count are usually normal in people with JRA.
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• Antinuclear antibody (ANA): Antinuclear antibody is just one of the antibodies that the body may produce in certain autoimmune diseases (called autoantibodies). As many as 25% of children with JRA have a positive ANA result. A positive ANA result is most common in children with pauciarticular disease. It is uncommon in children with systemic JRA. It is linked to an increased risk of eye involvement (uveitis). ANA is also more likely to be positive in conditions related to JRA (such as SLE or scleroderma) than in JRA. It is often used to rule out these conditions in a person with arthritis symptoms. Very high levels of ANA may increase the risk that the disease will progress to adult-type SLE. 
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• Rheumatoid factor (RF): Rheumatoid factor is actually a group of autoantibodies that occur in some people with RA, JRA, and related conditions. It is most often positive in children with polyarticular JRA and is rarely positive in children with systemic JRA. It is most often used to help determine which type of JRA a child has. Adolescents are more likely to have a positive RF result than younger children. In fact, many consider a positive RF result a sign of JRA progressing to adult-type RA.
  

Imaging studies

Your child’s health care provider may order x-rays or similar imaging studies. These images may help confirm the diagnosis or JRA or suggest other conditions that might cause similar symptoms.

• X-rays: X-rays give information about whether or not damage has occurred in the joint.
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• Bone scan: A bone scan may be necessary if the results of the workup do not support the diagnosis of JRA. A bone scan can detect inflammation in the bone and other abnormalities that do not show up well on x-ray.
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• MRI: MRI is similar to x-ray but is much more detailed and gives a better 3-dimensional view of the joint. It is especially useful if there has been a fall or accident that might have injured joints. It usually is not ordered unless the results of the workup do not point clearly to a diagnosis of JRA.
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• CT scan: CT scan also is similar to x-ray but provides much greater detail. It may be ordered when the results of the workup do not support the diagnosis of JRA. CT scan is particularly good at ruling out tumors and other bony abnormalities that could cause arthritis-like symptoms.
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• Dual-energy x-ray absorptiometry (DEXA) scan: DEXA scanning is used to measure bone density and can identify osteopenia or osteoporosis (loss of bone tissue) in children with polyarticular JRA.
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Other tests

Other tests may be ordered in specific circumstances. Most children with JRA do not require these tests.

• Arthrocentesis: This is sometimes called “joint aspiration.” It means the removal of a sample of synovial fluid (fluid from a joint cavity) for testing. It is usually done to rule out infections in the joints.
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• Synovial biopsy: An orthopedic surgeon uses a probe to remove a small amount of the synovial tissue from a joint. The tissue is examined under a microscope for clues as to what is causing synovial damage. This may be helpful to exclude other conditions that can cause symptoms similar to those of JRA.

The main goal of treatment in children with juvenile rheumatoid arthritis is help them live as normal a life as possible. To be successful, this treatment must address all aspects of the disease, including medical problems and complications, physical functioning, school performance, and social and emotional adjustment.

• Care requires the coordinated efforts of a team of professionals.
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• This care may be overseen by the child’s primary care provider, with consultation from a specialist in rheumatic diseases such as arthritis and similar conditions (a rheumatologist), preferably one who specializes in rheumatic diseases of children, as well as specialists in eye problems (ophthalmologist), skin problems (dermatologist), heart problems (cardiologist), digestive problems (gastroenterologist), kidney problems (nephrologist), lung problems (pulmonologist), and/or orthopedic surgery, if necessary.
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• Medical treatment is only part of the equation. The team also may include physical and occupational therapists and a psychologist or counselor. A social worker can help the family cope with the social, financial, and emotional aspects of the disease.
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• Although medication is the cornerstone of treatment of JRA, medication alone is unlikely to be successful if the child is not also receiving appropriate physical therapy, emotional counseling, and school assistance.

|Self-Care at Home|

Self-treatment is not encouraged in JRA. Without appropriate inflammation-stopping medical treatment, the inflammation in JRA progresses and becomes worse, increasing the risk of permanent damage to joints, eyes, and other body systems. You can take steps at home, however, to improve your child’s comfort and decrease his or her chance of becoming disabled with JRA.

• Encourage your child to be as active as possible. Bed rest is not part of the treatment of JRA except for children with severe systemic disease. Indeed, the more active the child, the better the long-term outlook. Swimming and water aerobics are ideal activities because they place no stress on the joints. Children may experience pain during routine physical activities and thus must be allowed to limit their own activities, particularly during physical education classes. A consistent physical therapy program, with attention to stretching exercises, pain avoidance, joint protection, and home exercises, can help ensure that a child with JRA is as active as possible.
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• Make sure your child is eating enough to maintain a healthy weight. Some children with JRA have little appetite. They need to be encouraged to eat enough calories to maintain a healthy weight and a proper energy level. A balanced diet that provides all required vitamins and minerals is essential. Give your child at least 3 servings of calcium-rich foods each day. While there is no evidence that this actually improves JRA, it does help keep bones strong and flexible. Make sure your child also gets enough magnesium and vitamin D to help keep bones healthy. Ask your child’s health care provider for information about diet and nutrition for your child. He or she can refer you to a dietitian if necessary.
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• Help your child learn techniques for dealing with the discomfort and pain of JRA. In many cases, combining pain-relieving medication (analgesics) with other techniques gives the best balance of pain relief with fewest unwanted side effects. Techniques such as biofeedback, progressive muscle relaxation, meditation, deep breathing, and guided imagery can help children overcome pain. Hot baths or showers, a warm bed, range-of-motion exercises, and hot packs can relieve morning stiffness. Some children respond better to cold packs than to warmth. A plastic bag of frozen vegetables makes a great ice pack.

|Medical Treatment|

The aim of treatment is to stop or slow down the progress of inflammation, thus relieving symptoms, improving function, and preventing joint damage and other complications. Specific objectives are to reduce joint swelling, stiffness, and pain; maintain full range of motion of all joints; and identify and treat complications early, when they can be stopped or reversed. The success of treatment is checked by regular physical examinations and interviews.
 
Medication is the foundation of treatment in JRA. The medications that work best in JRA reduce inflammation, which in turn reduces symptoms. Aggressive, early treatment is the best way to stop or slow the disease over the long run. Various classes of medications used in JRA are described here. 
 
Nonsteroidal anti-inflammatory drugs (NSAIDs) reduce inflammation, swelling, and pain. They work by blocking an enzyme called cyclo-oxygenase (COX), which promotes inflammation.

• These drugs are used to treat all types of JRA and are usually the first choice of treatment.
  
  
• An NSAID alone may be adequate in pauciarticular disease and mild cases of polyarticular disease.
  
  
• Children with more severe disease often require a second drug to be added to the NSAID. This is usually a drug from another class, since taking more than one NSAID does not help the disease and may cause severe side effects.
  
  
• It usually takes at least 4 weeks to see whether treatment with a specific NSAID is going to work.
  
  
• Aspirin is no longer a first choice in JRA because of its side effects. The side effects can be (but rarely are) serious, especially those in the digestive tract and liver.
  
  
• A newer generation of these drugs is called the COX-2 inhibitors. These drugs are much less likely than other NSAIDs to cause digestive side effects in adults. Rofecoxib (Vioxx) is a COX-2 inhibitor that was recently approved by the United States Food and Drug Administration (FDA) for treating JRA. However, on September 30, 2004, Merck & Co, Inc, announced a voluntary withdrawal of rofecoxib (Vioxx) from the US and worldwide market because of its association with an increased rate of cardiovascular events (including heart attacks and strokes) compared to that of placebo.
  
  
• Predicting which children will respond to a particular NSAID is impossible. Children who show no improvement after 1-2 months of treatment may benefit from changing to a different NSAID.
  
  
• Common side effects include nausea and vomiting, stomach pain, and anemia. Other side effects depend on the NSAID.

Disease-modifying antirheumatic drugs (DMARDs) are not a single class of drugs. Rather, they are a wide variety of different drugs that act in many different ways. Their main similarity is that they interfere in the immune processes that cause inflammation and JRA. DMARDs can slow or stop the progression of JRA and thus joint damage and disability.

• DMARDs may be given alone or in combination with other types of drugs. On the other hand, successful DMARD therapy may eliminate the need for other anti-inflammatory or analgesic medications.
  
  
• Many, but not all, work by stopping the autoimmune response; they are called “immunosuppressive drugs.”
  
  
• These drugs do not work for everyone with JRA, but they give substantial relief to many.
  
  
• DMARDs may not reach their full effect for several months. It is important that the child keeps taking the medication for at least that long before you decide that it is not working. Until the full action of a DMARD takes effect, your child’s health care provider may prescribe anti-inflammatory or analgesic medications as “bridging therapy” to reduce pain and swelling.
  
  
• These drugs have many potential side effects (which vary by drug). Children taking some of these drugs require regular blood tests to check for side effects.
  
  
• The immunosuppressive drugs impair the immune system’s ability to fight infections. Anyone taking one of these drugs must be very vigilant to watch for early signs of infection, such as fever, cough, or sore throat. Early treatment of infections can prevent more serious problems.
  
  
• These drugs have been shown to improve signs and symptoms (as well as quality of life) in most children with JRA.

Biologic response modifiers are a newer, specialized type of immunosuppressive drugs.

• These agents are carefully designed to block the actions of natural substances that are part of the immune response, such as tumor necrosis factor or interleukin-1. Therefore, these agents inhibit the autoimmune reaction that causes JRA.
  
  
• Blocking these substances reduces joint inflammation and thus relieves symptoms and improves the child’s overall condition.
  
  
• There are several different versions of these agents, and in some children JRA will get better with one version and not another.
  
  
• Although these agents slow down inflammation in a significant proportion of children with JRA, they do not usually lead to remission.
  
  
• These agents are expensive.
  
  
• It may take about 2-3 months to see whether a biologic agent is working in a specific individual.
  
  
• Children with an infection (especially tuberculosis), cancer now or in the recent past, or certain types of nervous system disorders cannot take these agents.
  
  
• These agents improve signs and symptoms and quality of life in many people with JRA.

Glucocorticoids (“steroids”), another group of immunosuppressive drugs, are very potent anti-inflammatory agents that block inflammation and other immune responses. All steroids work in the same way; they differ only in their potency and in the form in which they are given. They stop or slow joint damage and reduce symptoms.

• These drugs can be given as pills by mouth, into a muscle (intramuscularly), into a vein (intravenously), or in some cases as an injection directly into a joint.
  
  
• Steroids given in high doses can have many side effects. They tend to lose their effectiveness over time while still causing the same side effects. Furthermore, they can be given safely only for short periods—a few weeks or months. Therefore, these drugs are commonly used to bridge the gap while waiting for a DMARD to reach full effect.
  
  
• These agents are not for everyone. Your child’s health care provider will decide whether glucocorticoids are right for your child on the basis of his or her overall medical condition.
  
  
• In children these drugs are typically given at the lowest possible dose for the shortest possible time to avoid side effects.
  
  
• It is very important not to stop taking a glucocorticoid abruptly, as this can be dangerous. The only safe way to stop taking these medications is to gradually lower (“taper”) the dose. If your child seems to be having severe side effects, talk to his or her health care provider before stopping the drug.

Analgesics are drugs that reduce pain but do not affect inflammation, swelling, or joint destruction.

• Acetaminophen/paracetamol, tramadol, codeine, opiates, and a variety of other analgesic medications can be employed to reduce pain.
  
  
• Acetaminophen is sometimes used for children with mild JRA who cannot take NSAIDs because of hypersensitivity, ulcers, liver problems, or interactions with other drugs. At very high doses, however, this drug also can harm the liver. 
  
  
• These agents usually are given only with other medications.
  
  
• They should be given only under the supervision of your child’s health care provider.

Nondrug approaches include the following:

• Physical therapy helps preserve and improve range of motion, increase muscle strength, and reduce pain.
  
  
• Hydrotherapy involves exercising or relaxing in warm water. Being in water reduces most of the weight on the joints. The warmth relaxes the muscles and helps relieve pain.
  
  
• Relaxation therapy teaches techniques for releasing muscle tension, which helps relieve pain.
  
  
• Both heat and cold treatments can relieve pain and reduce inflammation. Some children’s pain responds better to heat and others to cold. Heat can be applied by ultrasound, microwaves, warm wax, or moist compresses. Most of these are done in the medical office, although moist compresses can be applied at home. Cold usually is applied by ice pack.
  
  
• Occupational therapy teaches your child ways to use his or her body efficiently to reduce stress on the joints. It also can help the child learn to decrease tension on the joints through the use of specially designed splints. Your child’s occupational therapist can help your child develop strategies for coping with daily life by adapting to his or her environment and using different assistive devices.

|Medications|

Nonsteroidal anti-inflammatory drugs

• Ibuprofen (Advil, Ibuprin, Motrin)
  
  
• Naproxen (Aleve, Naprelan, Naprosyn)
  
  
• Diclofenac (Cataflam, Voltaren)
  
  
• Indomethacin (Indocin)
  
  
• Tolmetin (Tolectin)
  
  
• Oxaprozin (Daypro)
  
  
• COX-2 inhibitors - Celecoxib (Celebrex), rofecoxib (Vioxx)

Disease-modifying antirheumatic drugs

• Methotrexate (Rheumatrex, Folex PFS): We do not know exactly how this drug works in treatment of inflammatory conditions. It relieves symptoms of inflammation such as pain, swelling, and stiffness. It may be given by injection if the oral form has little effect. Children taking methotrexate have to have regular blood tests to measure whether the drug is having any adverse effects on the liver or blood cells. It is the standard treatment for children with JRA in which joint damage is occurring. 
  
  
• Sulfasalazine (Azulfidine): This drug decreases inflammatory responses by an effect similar to that of aspirin or NSAIDs.
  
  
• Gold salts (aurothiomalate, auranofin [Ridaura]): These compounds contain very tiny amounts of the metal gold. They can be taken by mouth or as injections. We do not know why they stop inflammation. Apparently the gold infiltrates into immune cells and interferes with their activities. Gold compounds, once widely used in JRA, are now rarely used in this disease.
  
  
• Azathioprine (Imuran): This drug stops production of cells that are part of the immune response that causes JRA. Unfortunately, it also stops production of some other types of cells and thus can have serious side effects. It very strongly suppresses the entire immune system and thus leaves the person vulnerable to infections and other problems. It is used only in very severe cases of JRA that have not gotten better with other DMARDs.
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• Cyclosporin A (Neoral): This drug was developed for use in people undergoing organ transplantation. These people must have their immune system suppressed to prevent rejection of the transplant. Cyclosporin blocks an important immune cell and interferes with the immune response in several other ways. It is used most often in systemic JRA.
  
  
• Leflunomide (Arava): This drug blocks immune antibodies and reduces inflammation. It reduces symptoms and may even slow progression of JRA. This agent is not suitable for some people with kidney problems.
  
  

Biologic response modifiers

• Etanercept (Enbrel): This agent blocks the action of tumor necrosis factor, which in turn decreases inflammatory and immune responses. It is given by subcutaneous injection twice weekly. 
  
  
• Infliximab (Remicade): This antibody blocks the action of tumor necrosis factor. It is usually used in combination with methotrexate in children whose JRA does not respond to methotrexate alone. It is given by intravenous infusion every 6-8 weeks. 
  
  
• Adalimumab (Humira): This is another blocker of tumor necrosis factor. It reduces inflammation and slows or stops worsening of joint damage in fairly severe JRA. This agent is used mainly for people whose JRA has not responded to at least 2 DMARDs. It is given by subcutaneous injection every other week. 
  
  
• Anakinra (Kineret): This agent blocks the action of interleukin-1, which is partly responsible for the inflammation of JRA. This in turn blocks inflammation and pain. This agent usually is reserved for children whose JRA has not improved with DMARDs. It is given by subcutaneous injection daily. 
  
  
• Only etanercept has been fully studied in JRA. Clinical trials of the other biologic response modifiers are now being carried out to see whether these agents offer a benefit to children with JRA.
  
  

Glucocorticoids

• Prednisone (Deltasone, Meticorten, Orasone)
  
  
• Dexamethasone (Decadron)
  
  
• Methylprednisolone (Medrol)
  
  
• Betamethasone (Celestone)
  
  

Analgesics

• Acetaminophen (Tylenol, Feverall, Tempra)
  
  
• Tramadol (Ultram)
  
  

All medications have side effects, and the drugs used in JRA are no exceptions. Most studies of drug side effects are done in adults, and less is known about side effects in children. Side effects for a given drug may be much different in children than in adults. The medical professional who prescribes medication for a child with JRA should watch the child’s response very carefully and adjust the dose accordingly. The goal is to find the proper balance between improving the child’s condition and minimizing side effects.

|Surgery|

Surgery is not usually needed in JRA.

• Some children with persisting pauciarticular JRA, despite medical treatment, may benefit from steroid injections into a joint. This may be done by a rheumatologist, an orthopedic surgeon, or in some cases a child’s primary care provider.
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• Joint replacement (often of the hips, in patients with polyarticular JRA) usually is delayed until bone growth has completed.
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• At one time, part of the synovium was removed (synovectomy) in very severe cases to prevent joint damage. With improvements in medical treatment, this procedure is now rarely necessary.

|Other Therapy|

A variety of complementary approaches can be very effective in relieving pain. These include acupuncture and massage. These are not necessary, but may help your child feel more comfortable.

|Follow-up|

Whenever possible, children with juvenile rheumatoid arthritis should receive their care from a multidisciplinary center that specializes in pediatric rheumatologic care. However, such centers are not common and are mostly confined to large medical centers.
 
Children with JRA must be evaluated regularly by the provider supervising care of their disease. This provider will give referrals to appropriate specialists, who will evaluate the child for development of preventable and/or treatable complications. Care will continue throughout childhood and adolescence. Children who go on to develop adult-type RA require medical care for their entire lives.

|Prevention|

We do not know how to prevent JRA. Following recommended treatment is the best way to prevent worsening of the disease. If left untreated, JRA will continue to worsen and eventually can lead to joint damage and disability, and possibly other serious complications.

|Outlook|

In most cases, JRA responds slowly and gradually to appropriate treatment.

• Children with pauciarticular JRA are most likely to have progressive improvement in symptoms, range of motion, and function. In many, the symptoms and signs will disappear completely (remission). Many have little disability and a high level of functioning.
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• A small number of patients with pauciarticular JRA develop aggressive arthritis confined to a single joint. These patients require more intensive medical treatment and physical therapy.
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• Some patients with polyarticular JRA have a rapid response to treatment with few residual symptoms. Most, however, have prolonged courses, requiring frequent adjustments in medical and nonmedical therapy. Some have significant loss of function and benefit from extensive physical and occupational therapy. Some have problems with active joint inflammation through adulthood.
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Complications

• Systemic JRA
  
  
  • Pericarditis: Unexplained shortness of breath is the most common symptom.
    
    
  • Anemias and similar blood disorders
    
    
  • Inflammation of the arteries in the hands and/or feet: This may impair blood circulation and cause serious damage to fingers and/or toes.
  
  
• Pauciarticular JRA
  
  
  • Knee contractures: The knee stiffens in the bent position.
    
    
  • Uveitis: This complication is often without symptoms. It is most common in young girls who have a positive ANA result. It can lead to a decrease in vision.
    
    
  • Leg length discrepancy
  
  
• Polyarticular JRA
  
  
  • Skeletal abnormalities
    
    
  • Involvement of the spine in the neck: The child may have difficulty bending the neck forward.

|Support Groups and Counseling|

Living with the effects of JRA can be difficult. Both you and your child will sometimes feel frustrated, perhaps even angry or resentful. Sometimes it helps to have someone to talk to. A counselor or psychologist can help the child and family members deal with these negative feelings and develop positive, helpful attitudes.
 
Support groups also can help. Support groups consist of people in the same situation you are in. They come together to help each other and to help themselves. Support groups provide reassurance, motivation, and inspiration. They help you see that your situation is not unique, and that gives you power. They also provide practical tips on coping with your child’s disease.
 
There are support groups for the child with JRA, for the brothers and sisters, and for the parents. Support groups meet in person, on the telephone, or on the Internet. To find a support group that works for you, ask your health care provider or contact the following organizations or look on the Internet. If you do not have access to the internet, go to the public library.

• American Juvenile Arthritis Organization – (404) 872-7100 or (800) 283-7800

American Juvenile Arthritis Organization
1330 West Peachtree Street
Atlanta, FA 30309
(404) 872-7100 or (800) 283-7800
 
Arthritis Foundation
P.O. Box 7669
Atlanta, GA 30357-0669
(800) 283-7800
 
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Information Clearinghouse
National Institutes of Health
1 AMS Circle
Bethesda, MD 20892-3675
(301) 495-4484 or toll free (877) 226-4267
 
American College of Rheumatology/Association of Rheumatology Health Professionals
1800 Century Place, Suite 250
Atlanta, GA 30345-4300
(404) 633-3777

|Web Links|

American College of Rheumatology/Association of Rheumatology Health Professionals
 
Arthritis Foundation, American Juvenile Arthritis Organization
 
National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)

Media file 1: Active pauciarticular arthritis. Notice the swelling above the knee. Image courtesy of Barry L. Myones, MD.

Media type:  Photo

Media file 2: Active polyarticular arthritis. Notice the swelling of only certain finger joints. Image courtesy of Barry L. Myones, MD.

Media type:  Photo

Media file 3: Wrist x-rays of active polyarticular arthritis (same person as Image 2). X-ray shows severe loss of cartilage, bone erosion, and joint fixation and narrowing. Image courtesy of Barry L. Myones, MD.

Media type:  X-RAY

Media file 4: Close-up of the affected joints in a person with active polyarticular arthritis (same person shown in Images 2 and 3). Image courtesy of Barry L. Myones, MD.

Media type:  Photo

Media file 5: Inactive polyarticular arthritis. Long-term symptoms of polyarticular disease include partial joint dislocation (subluxation) of both wrists and thumbs, joint contractures, boney overgrowth, and finger deformities (eg, swan-neck or boutonniere deformities). Image courtesy of Barry L. Myones, MD.

Media type:  Photo

Media file 6: Hand and wrist x-rays of inactive polyarticular arthritis (same person shown in Image 5). Long-term symptoms of polyarticular disease include decreased bone density around joints, bone fusion, accelerated bone age, narrowed joint spaces, boutonniere deformities (at left third and fourth interphalangeal joints), and partial dislocation (subluxation) of joints. Image courtesy of Barry L. Myones, MD.

Media type:  X-RAY

Media file 7: Eye inflammation (chronic anterior uveitis). Note the weblike attachments of the pupillary margin to the anterior lens capsule of the right eye. This patient has a positive antinuclear antibodies (ANAs) and initially had a pauciarticular course of her arthritis. She now has polyarticular involvement but no active uveitis. Image courtesy of Barry L. Myones, MD.

Media type:  Photo

Media file 8: One set of suggested algorithms for the treatment of patients with juvenile rheumatoid arthritis (JRA). This treatment is not standardized. Treatment of JRA is based on experience and observation, and it can be controversial.

Media type:  Illustration

JRA, rheumatoid arthritis, RA, arthritis, joint inflammation, juvenile arthritis, synovium, joints, antibodies, autoantibodies, autoimmune disease, eye inflammation, immune response, inflammation, inflammatory disease, iritis, joint disease, joint pain, juvenile idiopathic arthritis, systemic JRA, pauciarticular JRA, polyarticular JRA, rheumatic disease, Still disease, Still’s disease, uveitis, juvenile rheumatoid arthritis

Author: Michael Miller, MD, Associate Professor of Pediatrics, Division of Immunology and Rheumatology, Children's Memorial Hospital of Chicago, Northwestern University Medical School.

Editors: Kristine M Lohr, MD, Associate Chief, Program Director, Professor, Department of Internal Medicine, Division of Rheumatology, University of Tennessee School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, ; Arthur Weinstein, MD, Professor of Medicine, Georgetown University; Associate Chairman, Department of Medicine, Director, Section of Rheumatology, Washington Hospital Center.