Juvenile rheumatoid arthritis

Juvenile chronic polyarthritis; JRA; Still's disease; Juvenile idiopathic arthritis

Last reviewed: June 28, 2011.

Juvenile rheumatoid arthritis (JRA) is a term used to describe a common type of arthritis in children. It is a long-term (chronic) disease resulting in joint pain and swelling.

Causes, incidence, and risk factors

The cause of JRA is not known. It is thought to be an autoimmune illness. This means the body's immune system mistakenly attacks and destroys healthy body tissue. JRA usually occurs before age 16. Symptoms may start as early as 6 months old. It is divided into several types: Systemic (bodywide) JRA involves joint swelling or pain, fevers, and rash. It is the least common type. Polyarticular JRA involves many joints. This form of JRA may turn into rheumatoid arthritis. It may involve five or more large and small joints of the legs and arms, as well as the jaw and neck. Pauciarticular JRA involves four or less joints, most often the wrists, or knees. It also affects the eyes.

Symptoms
Symptoms of JRA may begin with a swollen joint, limping, a spiking fever, or a new rash. Symptoms can include: Joint stiffness and pain Limited range of motion Warm, swollen, or red joints A child may stop using an affected limb or may limp

Body-wide JRA symptoms: Fever, usually high fevers every day Rash (trunk and extremities) that comes and goes with the fever Pale skin Looks sick Swollen lymph nodes (glands)

JRA can also cause eye problems called uveitis, iridocyclitis, or iritis. There may be no symptoms, or the person may have: Red eyes Eye pain, which may get worse when looking at light (photophobia)

Vision changes

Signs and tests

The physical examination may show swollen, warm, and tender joints that hurt to move. The child may have a rash. Other signs include: Swollen liver Swollen spleen Swollen lymph nodes

Blood tests that may be done include: Rheumatoid factor Erythrocyte sedimentation rate (ESR) ANA Complete blood count (CBC) HLA antigens for HLA B27

Any or all of these blood tests may be normal in patients with JRA. The health care provider may place a small needle into a swollen joint to remove fluid. This can help to find the cause of the arthritis and help relieve pain, too. Sometimes, the health care provider will inject steroids into the joint to help reduce swelling. Other tests that may be done include: X-ray of a joint Bone scan X-ray of the chest ECG Eye exam by an ophthalmologist (should be done on a regular basis, even if there are no eye symptoms)

Treatment
When only a small number of joints are involved, nonsteroidal anti-inflammatory drugs (NSAIDs) such asibuprofen or naproxen may be enough to control symptoms. Corticosteroids may be used for more severe flare-ups to help control symptoms. Children who have arthritis in many joints, or who have fever, rash, and swollen glands may need other medicines. These medicines are called disease-modifying antirheumatic drugs (DMARDs). They can decrease or prevent swelling in the joints or body. DMARDs include: Methotrexate Biologic drugs, such as such as etanercept, infliximab, and related drugs

It is important for children with JRA to stay active and keep their muscles strong. Exercise will help keep their muscles and joints strong and mobile. Walking, bicycling, and swimming may be good activities.

Children should learn to warm up before exercising. Talk to the doctor or physical therapist about exercises to do when your child is having pain.

Support and help for children who have sadness or anger about their arthritis is also very important. Some children with JRA may need surgery, including joint replacement.

Expectations (prognosis)
Long periods with no symptoms are more common in those who have only a small number of joints involved. Many children with JRA eventually go into remission with very little loss of function and joint damage. The greater the number of joints affected, the more severe the disease and the less likely that the symptoms will eventually go into total remission. Children with JRA who have many joints involved, or who have a positive rheumatoid factor are more likely to have chronic pain, disability, and poor school attendance. The greater the number of joints affected, the more severe the disease and the less likely that the symptoms will eventually go into total remission. For additional information and resources, see: Arthritis support group

Complications
Wearing away or destruction of joints (can occur in patients with more severe JRA) Slow rate of growth Uneven growth of an arm or leg Loss of vision or decreased vision from chronic uveitis (this problem may be severe, even when the arthritis is not very severe) Anemia Swelling around the heart (pericarditis) Chronic pain, poor school attendance

Calling your health care provider

Call for an appointment with your health care provider if: You notice symptoms of juvenile rheumatoid arthritis Symptoms get worse or do not improve with treatment New symptoms develop

Prevention
There is no known prevention for JRA.

Definition
By Mayo Clinic staff Juvenile rheumatoid arthritis, also known as juvenile idiopathic arthritis, is the most common type of arthritis in children under the age of 16. Juvenile rheumatoid arthritis causes persistent joint pain, swelling and stiffness. Some children may experience symptoms for only a few months, while others have symptoms for the rest of their lives. Some types of juvenile rheumatoid arthritis can cause serious complications, such as growth problems and eye inflammation. Treatment of juvenile rheumatoid arthritis focuses on controlling pain, improving function and preventing joint damage.

Symptoms
By Mayo Clinic staff The most common signs and symptoms of juvenile rheumatoid arthritis are: Pain. While your child might not complain of joint pain, you may notice that he or she limps especially first thing in the morning or after a nap. Swelling. Joint swelling is common but is often first noticed in larger joints like the knee. Stiffness. You might notice that your child appears clumsier than usual, particularly in the morning or after naps. Juvenile rheumatoid arthritis can affect one joint or many. In some cases, juvenile rheumatoid arthritis affects the entire body causing swollen lymph nodes, rashes and fever. Like other forms of arthritis, juvenile rheumatoid arthritis is characterized by times when symptoms flare up and times when symptoms disappear. When to see a doctor Take your child to the doctor if he or she has joint pain, swelling or stiffness for more than a week especially if he or she also has a fever.

Causes
By Mayo Clinic staff

Juvenile rheumatoid arthritis occurs when the body's immune system attacks its own cells and tissues. It's unknown why this happens, but both heredity and environment seem to play a role. Certain gene mutations may make a person more susceptible to environmental factors such as viruses that may trigger the disease.

Risk factors
By Mayo Clinic staff In general, juvenile rheumatoid arthritis is more common in girls.

Complications
By Mayo Clinic staff Several serious complications can result from juvenile rheumatoid arthritis. But keeping a careful watch on your child's condition and seeking appropriate medical attention can greatly reduce the risk of these complications: Eye problems. Some forms of juvenile rheumatoid arthritis can cause eye inflammation (uveitis). If this condition is left untreated, it may result in cataracts, glaucoma and even blindness. Eye inflammation frequently occurs without symptoms, so it's important for children with juvenile rheumatoid arthritis to be examined regularly by an ophthalmologist. Growth problems. Juvenile rheumatoid arthritis can interfere with your child's growth and bone development. Some medications used to treat juvenile rheumatoid arthritis, mainly corticosteroids, also can inhibit growth.

Tests and diagnosis

By Mayo Clinic staff Diagnosis of juvenile rheumatoid arthritis can be difficult because joint pain can be caused by many different types of problems. No single test can confirm a diagnosis, but tests can help rule out some other conditions that produce similar signs and symptoms. Blood tests Some of the most common blood tests for suspected cases of juvenile rheumatoid arthritis include: Erythrocyte sedimentation rate (ESR). Sedimentation rate is the speed at which your red blood cells settle to the bottom of a tube of blood. An elevated rate can indicate inflammation. Measuring the ESR may be used to rule out other conditions, to help classify the type of juvenile rheumatoid arthritis and to determine the degree of inflammation.

C-reactive protein. This blood test also measures levels of general inflammation in the body but on a different scale than the ESR.

Anti-nuclear antibody. Anti-nuclear antibodies are proteins commonly produced by the immune systems of people with certain autoimmune diseases, including arthritis.

Rheumatoid factor. This antibody is commonly found in the blood of adults who have rheumatoid arthritis.

Cyclic citrullinated peptide (CCP). Like the rheumatoid factor, the CCP is another antibody that may be found in the blood of adults with rheumatoid arthritis. In many children with juvenile rheumatoid arthritis, no significant abnormality will be found in these blood tests. Imaging scans X-rays may be taken to exclude other conditions, such as:

Fractures Tumors Infection Congenital defects X-rays may also be used from time to time after the diagnosis to monitor bone development and to detect joint damage.

Treatments and drugs

By Mayo Clinic staff Treatment for juvenile rheumatoid arthritis focuses on helping your child maintain a normal level of physical and social activity. To accomplish this, doctors may use a combination of strategies to relieve pain and swelling, maintain full movement and strength, and prevent complications. Medications For some children, pain relievers may be the only medication needed. Other children may need help from medications designed to limit the progression of the disease. Typical medications used for juvenile rheumatoid arthritis include:

Nonsteroidal anti-inflammatory drugs (NSAIDs). These medications, such as ibuprofen (Advil, Motrin, others) and naproxen (Aleve), reduce pain and swelling. Stronger NSAIDs are available by prescription. Side effects include stomach upset and liver problems.

Disease-modifying antirheumatic drugs (DMARDs). Doctors use these medications when NSAIDs alone fail to relieve symptoms of joint pain and swelling. They may be taken in combination with NSAIDs and are used to slow the progress of juvenile rheumatoid arthritis. Commonly used DMARDs for children include methotrexate (Trexall) and sulfasalazine (Azulfidine). Side effects may include nausea and liver problems.

Tumor necrosis factor (TNF) blockers. TNF blockers such as etanercept (Enbrel) and adalimumab (Humira) can help reduce pain, morning stiffness and swollen joints. But these types of drugs increase the risk of infections. There may also be a mild increase in the chance of getting some cancers, such as lymphoma.

Immune suppressants. Because juvenile rheumatoid arthritis is caused by an overactive immune system, medications that suppress the immune system can help. Examples include abatacept (Orencia), rituximab (Rituxin), anakinra (Kineret) and tocilizumab (Actemra). Immune suppressants increase the risk of infections and, rarely, some types of cancer.

Corticosteroids. Medications such as prednisone may be used to control symptoms until a DMARD takes effect or to prevent complications, such as inflammation of the sac around the heart (pericarditis). Corticosteroids may be administered by mouth or by injection directly into a joint. But these drugs can interfere with normal growth and increase susceptibility to infection, so they generally should be used for the shortest possible duration. Therapies Your doctor may recommend that your child work with a physical therapist to help keep joints flexible and maintain range of motion and muscle tone. A physical therapist or an occupational therapist may make additional recommendations regarding the best exercise and protective equipment for your child. A therapist may also recommend that your child make use of joint supports or splints to help protect joints and keep them in a good functional position. Surgery In very severe cases of juvenile rheumatoid arthritis, surgery may be needed to improve the position of a joint.

Lifestyle and home remedies

By Mayo Clinic staff

Controlling Your Arthritis

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Sign up now Caregivers can help children learn self-care techniques that help limit the effects of juvenile rheumatoid arthritis. Techniques include: Getting regular exercise.Exercise is important because it promotes both muscle strength and joint flexibility. Swimming is an excellent choice because it places minimal stress on joints. Applying cold or heat. Stiffness affects many children with juvenile rheumatoid arthritis, particularly in the morning. Although some children respond well to cold packs, most children prefer a hot pack or a hot bath or shower. Eating well. Some children with arthritis have poor appetites. Others may gain excess weight due to medications or physical inactivity. A healthy diet can help maintain an appropriate body weight. Adequate calcium in the diet is important because children with juvenile rheumatoid arthritis are at risk of developing weak bones (osteoporosis) due to the disease, the use of corticosteroids, and decreased physical activity and weight bearing.