What is JRA?Juvenile Rheumatoid Arthritis is an inflammation of the joints that is characterized by swelling, heat, and pain. Nearly 300,000 children in the United States have some sort of arthritis. Research indicates that it is an autoimmune disease. In autoimmune diseases, white blood cells lose the ability to tell the difference between the body's own healthy cells and harmful invaders like bacteria and viruses. The immune system, which is supposed to protect the body from these harmful invaders, instead releases chemicals that can damage healthy tissues and cause inflammation and pain. Alternative Names:Juvenile chronic polyarthritis; JRA; Still's disease; Juvenile idiopathic arthritis
Types of JRA? There are 3 major types of juvenile rheumatoid arthritis: Polyarticular arthritis affects more girls than boys. Symptoms include swelling or pain in 5 or more joints. The small joints of the hands are affected as well as the weight-bearing joints such as the knees, hips, ankles, feet, and neck. In addition, a low-grade fever may appear, as well as bumps or nodules on the body on areas subjected to pressure from sitting or leaning. Pauciarticular JRA affects 4 or fewer joints. Symptoms include pain, stiffness, or swelling in the joints. The knee and wrist joints are the most commonly affected. An inflammation of the iris (the colored area of the eye) may occur with or without active joint symptoms. This inflammation, called iridocyclitis or iritis or uveitis, can be detected early by an ophthalmologist. Systemic JRA affects the whole body. Symptoms include high fevers that often increase in the evenings and then may suddenly drop to normal. During the onset of fever, the child may feel very ill, appear pale, or develop a rash. The rash may suddenly disappear and then quickly appear again. The spleen and lymph nodes may also become enlarged. Eventually many of the body's joints are affected by swelling, pain, and stiffness. What are the symptoms of JRA?The most common symptom of all types of JRA is persistent joint swelling, pain, and stiffness that typically is worse in the morning or after a nap. The pain may limit movement of the affected joint although many children, especially younger ones, will not complain of pain. JRA commonly affects the knees and joints in the hands and feet. One of the earliest signs of JRA may be limping in the morning because of an affected knee. Besides joint symptoms, children with systemic JRA have a high fever and a light skin rash. The rash and fever may appear and disappear very quickly. Systemic JRA also may cause the lymph nodes located in the neck and other parts of the body to swell. In some cases (less than half), internal organs including the heart and, very rarely, the lungs may be involved.Eye inflammation is a potentially severe complication that sometimes occurs in children with pauciarticular JRA. Eye diseases such as iritis and uveitis often are not present until some time after a child first develops JRA. Typically, there are periods when the symptoms of JRA are better or disappear (remissions) and times when symptoms are worse (flare-ups). JRA is different in each child--some may have just one or two flare-ups and never have symptoms again, while others experience many flare-ups or even have symptoms that never go away. Some children with JRA may have growth problems. Depending on the severity of the disease and the joints involved, growth in affected joints may be too fast or too slow, causing one leg or arm to be longer than the other. Overall growth may also be slowed. Doctors are exploring the use of growth hormones to treat this problem. JRA also may cause joints to grow unevenly or to one side. How is JRA diagnosed?Doctors usually suspect JRA, along with several other possible conditions, when they see children with persistent joint pain or swelling, unexplained skin rashes and fever, or swelling of lymph nodes or inflammation of internal organs. A diagnosis of JRA also is considered in children with an unexplained limp or excessive clumsiness.No one test can be used to diagnose JRA. A doctor diagnoses JRA by carefully examining the patient and considering the patient's medical history, the results of laboratory tests, and x rays that help rule out other conditions. Symptoms--One important consideration in diagnosing JRA is the length of time that symptoms have been present. Joint swelling or pain must last for at least 6 weeks for the doctor to consider a diagnosis of JRA. Because this factor is so important, it may be useful to keep a record of the symptoms, when they first appeared, and when they are worse or better. Laboratory tests--Laboratory tests, usually blood tests, cannot by themselves provide the doctor with a clear diagnosis. But these tests can be used to help rule out other conditions and to help classify the type of JRA that a patient has. Blood may be taken to test for RF and ANA, and to determine the erythrocyte sedimentation rate (ESR).ANA is found in the blood more often than RF, and both are found in only a small portion of JRA patients. The RF test helps the doctor tell the difference among the three types of JRA. ESR is a test that measures how quickly red blood cells fall to the bottom of a test tube. Some people with rheumatic disease have an elevated ESR or "sed rate" (cells fall quickly to the bottom of the test tube), showing that there is inflammation in the body. Not all children with active joint inflammation have an elevated ESR. X rays--X rays are needed if the doctor suspects injury to the bone or unusual bone development. Early in the disease, some x rays can show cartilage damage. In general, x rays are more useful later in the disease, when bones may be affected.Other diseases--Because there are many causes of joint pain and swelling, the doctor must rule out other conditions before diagnosing JRA. These include physical injury, bacterial or viral infection, Lyme disease, inflammatory bowel disease, lupus, dermatomyositis, and some forms of cancer. The doctor may use additional laboratory tests to help rule out these and other possible conditions. Treatments for JRAEarly diagnosis and appropriate treatment provides children with the best possible opportunity for a favorable outcome. Your child's treatment will be based on the kind of arthritis he/she has and on their specific symptoms. The goals of any treatment program for juvenile arthritis are: control inflammation, relieve pain, prevent or control joint damage and maximize joint and body function. MedicationsThe immediate goal of drug therapy is to reduce inflammation, relieve pain and swelling, and maximize function. Long-range goals are to alter the progress of the disease and the prevent damage to bone, cartilage and soft tissues such as muscles, tendons and joint capsules. It must be remembered that medication dosages in children must be carefully calculated based on their weight and body size. Medication doses should be adjusted regularly as the child grows to make sure the proper dosages are used. In addition, it is well known that children metabolize some medications differently than do adults. Therefore, drug dosages may be much higher than one would expect based just on the child's smaller body size. Side effects may be different in children, or occur rarely compared to adults taking the same medication. The following medications may be used to treat children with arthritis and related conditions. NSAIDs Nonsteroidal anti-inflammatory drugs (NSAIDs) are the first line of medication used in juvenile arthritis and are the mainstay of the initial therapy. NSAIDs must be taken for at least three to four weeks to tell whether they are helping control pain and inflammation. Laboratory tests may be done a few times a year to monitor medication side effects. These medications come in liquid or pill form and are taken from one to four times per day, depending on the drug prescribed. Some common NSAIDs on the market approved for children include: ibuprofen, naproxen, tolmentin, aspirin, choline magnesium trisalicylate and indomethacin. Possible side effects of NSAIDs include: occasional stomach pain, nausea and vomiting; anemia; headache; and uncommonly, blood in the urine; fluid retention; thinning and scarring of the skin (especially with naproxen); difficulty concentrating; and rarely, stomach ulcer. Aspirin Aspirin is a rarely prescribed NSAID these days. If chosen by your doctor, it may be used to control joint pain and swelling and to reduce fever just like the other NSAIDs. It is prescribed in large amounts and is given three or four times a day. Young children should not suck or chew on the aspirin because this may damage the chewing surfaces of the teeth and irritate the gums. Instead, try 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 of aspirin include stomach pains or stomach bleeding; toxic reactions can occur from too a high dose built up in your child's system (rapid or deep breathing, ringing in the ears, decrease in hearing, drowsiness, nausea, vomiting, irritability, unusual behavior and black stools). A rare complication called Reye's syndrome can occur in children who have the chicken pox or the flu and are taking aspirin. Symptoms include frequent vomiting, very painful headaches, unusual behavior, extreme tiredness and confusion. The different NSAIDs should never be combined together without your doctor's instructions. Slow Acting Anti-Inflammatory DrugsThese drugs do not relieve pain or inflammation right away; instead, they are given to change the progress of joint disease (such as joint erosions or cartilage and bone destruction) weeks to months after therapy is begun. Therefore, they are referred to as slow-acting anti-rheumatic drugs (SAARDs) or disease modifying anti-rheumatic drugs (DMARDs). These drugs are often used in combination with NSAIDs. Because they are more powerful medications, children will need to have more frequent laboratory tests for monitoring of possible side effects. Some of these medications are described below. Hydroxychloroquine Hydroxychloroquine (Plaquenil) is a pill used to control joint pain and swelling. It may take 3 to 6 months to work. While not helpful in all cases, it may be useful when methotrexate or gold have not been completely effective and can be used alone or in combination with other drugs. Side effects of hydroychloroquine include upset stomach, skin rash and a rare complication, eye damage. A child who takes this drug should have his/her eyes examined at least every six months by an ophthalmologist familiar with this medication. Sulfasalazine Sulfasalazine (Azulfadine) is given in pill form. This medication helps the joint pain, stiffness and swelling. It can sometimes have more side effects in children with systemic onset JRA, so should be used with caution in this type of JRA. It takes 6-12 weeks to work. Side effects may include stomach upset, achiness, diarrhea, dizziness, headache, light sensitivity, itching, appetite loss, liver abnormalities, lowered blood count, nausea, vomiting or rash. Blood work is checked within a few weeks of starting this medication then every few months to check for these changes.
Gold Compounds Gold compounds (Auranofin, Ridaura, Myochrysine and Solganol) are used to ease morning stiffness and control joint swelling and pain. The oral form is taken daily. Injections are usually given every week for five or six months, then one or twice a month for as long as necessary. These medications are only occasionally used in JRA any more. They take 3-6 months to work. Side effects may include skin rash, mouth sores, kidney problems, a low blood count or anemia. Blood and urine tests are checked every 1 to 4 weeks while taking gold compounds.
Penicillamine Penicillamine (DePen and Cuprimine) is given in pill form. This medication is rarely used for JRA but more often for scleroderma, an arthritis-related condition. It takes up to 6 months to work. Side effects include diarrhea, skin rash, hives or itching, low blood counts, mouth sores, nausea or vomiting, stomach pain, loss of taste or appetite, swollen glands, unusual bleeding or bruising, or weakness. Immune System Medications
Methotrexate Methotrexate (Rbeumatrex) is given weekly either orally as a liquid or in pill form, or by injection. It is one of the most commonly prescribed SAARDs for children with JRA. It can help the arthritis as well as the systemic illness in many children. It can help control uveitis in more severe cases. It takes 4-8 weeks to work. Few side effects have been reported at the low doses at which methotrexate is usually prescribed (typically 7.5 to 25 mg a week), but regular laboratory monitoring is still important. Blood tests are usually checked every month at first then every 6-8 weeks later on. This is also a cancer chemotherapy drug but the dosages used in children with JRA are much lower. Therefore, the side effects are less frequent. Side effects may include nausea, mouth sores, moodiness, diarrhea, low white blood cell count, lung irritation, infections and liver irritation. Avoid all alcohol intake and smoking while on this medication. Azathioprine Azathioprine (Imuran) is given in pill form. This is an immune system suppressing medication. It is not commonly used to treat JRA. It is saved for children that have failed or cannot take methotrexate. It takes about 3 months to work. Side effects can include cough, fever and chills, loss of appetite, nausea or vomiting, skin rash, unusual bleeding or bruising, unusual tiredness or weakness, or possibly, sterility. Cyclophosphamide Cyclophosphamide (Cytoxan) is given in pill form daily or intravenously in a single dose, usually monthly. This drug is rarely used for JRA but often given in children with systemic lupus erythematosus, an arthritis-related condition. It may take several months to work. Side effects include blood in the urine or burning on urination, confusion or agitation, cough, dizziness, fever and chills, infertility, loss of appetite, nausea or vomiting, unusual bleeding or bruising, unusual tiredness or weakness, and an increased risk for cancer. Cyclosporine Cyclosporine (Sandimmune) is given in liquid or pill form. This drug helps the joint inflammation as well as the systemic symptoms of systemic onset JRA. It may take a 1-2 months to work. Blood levels are often checked to determine the best dosage for your child. Side effects include bleeding, tender or enlarged gums; fluid retention; high blood pressure; increased hair growth; kidney problems; loss of appetite; or trembling or shaking of hands.
Biologic Agents Biological agents are a new class of medications made of synthetic proteins. These drugs may be made of antibodies that block high levels of inflammatory proteins in patients with arthritis. The drugs available include etanercept (Enbrel) which blocks the protein TNF, and was approved in 1998 by the FDA for RA treatment in adults, and in 1999 for the treatment of JRA. Infliximab (Remicade) is another anti-TNF medication that is approved to treat RA and has begun testing in JRA. Intravenous immunoglobulin (IVIG) is used to treat several childhood rheumatic diseases. It is usually given intravenously once a month. It is sometimes used as part of the treatment of systemic JRA. Side effects include the risk of allergic reactions, headaches, stomachache and flu-like symptoms. Researchers are developing other experimental biologic therapies that are aimed at specific proteins to control a variety of different diseases. Glucocorticoid Drugs Glucocorticoid medications (Dexamethasone, Methylprednisolone, Cortef, Prednisolone and Prednisone) are the most potent anti-inflammatory agents. These drugs are used to treat a variety of rheumatic diseases when the disease is severe or has not responded to other drugs. Sometimes glucocorticoids are used for a few weeks until other slower medications can become effective in controlling the arthritis. Because of their many side effects, glucocorticoids must be used with caution. If these drugs are prescribed, the lowest possible dose should be used for the shortest length of time. Usually the drug is given by mouth as a pill or liquid. It can also be given as an injection into the joint itself, or into a muscle, or vein (IV). Possible side effects of these medications include high blood pressure, osteoporosis (brittle bones), Cushing's syndrome (weight gain, moon face, thin skin, muscle weakness) cataracts, slowed growth rate, reduced resistance to infection, sudden mood swings, increased appetite and weight gain, or increased risk for ulcers. Many of these side effects occur only when the dose is more than 7.5 to 10 mg a day (dose for an average size adult who is taking prednisone). Glucocorticoids should never be stopped suddenly if they have been taken for more than a month. Your child's own natural gluco-corticoids may not be produced enough after using these drugs for a month or more. A schedule to slowly discontinue or taper these medications will be given to you by your doctor and will prevent this problem. A child taking glucocorticoids should wear a medical alert bracelet or necklace. This will alert emergency medical personnel to give extra medication if the child has surgery or is seriously injured.
AnalgesicsAnalgesics (acetaminophen [Tylenol, Panadol], tramadol [Ultram]) do not relieve inflammation, but provide pain relief. They should be taken only under a physician's advice in conjunction with other medications. Acetaminophen has few side effects when taken in small doses; very high doses (overdoses) can cause liver damage. Side effects of tramadol may include dizziness, nausea, constipation, headache and sleepiness. Self careYou 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…
- Make sure your child is eating enough to maintain a healthy weight. Some children with JRA have little appetite…
- 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..
Non-drug therapiesNon-drug 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.Complementary and alternative medicine. Many adults seek alternative ways of treating arthritis, such as special diets or supplements. Although these methods may not be harmful in and of themselves, no research to date shows that they help. Some people have tried acupuncture, in which thin needles are inserted at specific points in the body. Others have tried glucosamine and chondroitin sulfate, two natural substances found in and around cartilage cells, for osteoarthritis of the knee. Coping skillsFamily members can play critical roles in helping a child cope with juvenile rheumatoid arthritis. As a parent, you may want to try the following:Treat your child, as much as possible, like other children in your family.Allow your child to express anger about having juvenile rheumatoid arthritis. Explain that JRA isn't caused by anything he or she did.Encourage your child to participate in physical activities, keeping in mind the recommendations of your child's doctor and physical therapist.Work with your child's teachers and school administrators to make any necessary modifications in his or her schedule or responsibilities. These modifications may include giving your child extra time to move from class to class, providing him or her with an extra set of textbooks so that these books won't need to be carried home, and making arrangements for assignments to be sent home when your child misses school because of his or her condition. Also think about how you can cope with the challenges of raising a child who has juvenile rheumatoid arthritis. Connecting with other parents in similar circumstances and tapping into community resources may help. The Arthritis Foundation has a council called the American Juvenile Arthritis Organization (AJAO) devoted to serving the special needs of children with arthritis and their families. The organization sponsors support groups and conferences. What is the outlook for people with JRA?
JRA affects each child differently. For some, the disease is mild and easy to control, with only one or two joints affected. For others, JRA may involve many joints, and the symptoms may be more severe and may last longer. In general, however, the outlook is positive for children with JRA. Fifty percent to 75 percent of children with JRA experience complete remissions, or the disappearance of symptoms. Early detection and treatment may help to control inflammation, prevent joint damage and maintain your child’s ability to function. What complications are associated with JRA?
Untreated, JRA can lead to:Loss of vision or decreased vision due to iridocyclitis (inflammation of the iris)Permanent damage to jointsChronic arthritisInterference with a child’s bones and growthInflammation of the membranes surrounding the heart (pericarditis) or lungs (pleuritis) Can JRA be prevented?
At this time, there is no known way to prevent JRA.
© 2006 Waynette Porter