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Home > Disease & Condition > S > Still's Disease / Juvenile Rheumatoid Arthritis

Still's Disease / Juvenile Rheumatoid Arthritis

Definition :
Arthritis is usually associated with adults. But children can be affected by almost all of the types of arthritis that adults can have. Juvenile rheumatoid arthritis (JRA) — a chronic condition causing joint inflammation for at least six weeks in a child 16 years of age or younger — is the most common type of arthritis in children. In most cases juvenile rheumatoid arthritis is not a lifelong disorder, and the signs and symptoms may fade after several months or years.

Still, juvenile rheumatoid arthritis can be complicated. The term "juvenile rheumatoid arthritis" is actually an umbrella term for a group of conditions. The conditions are classified according to the number of joints affected, the signs and symptoms, and the results of blood tests.

Treatment of juvenile rheumatoid arthritis focuses on preserving physical activity to maintain full joint movement and strength.

Causes :
Doctors believe that juvenile rheumatoid arthritis is an autoimmune disorder. This means that the body attacks its own cells and tissues. It's unknown why this happens, but both heredity and environment seem to play a role.

It may be that a virus or bacterium triggers the development of juvenile rheumatoid arthritis in children with certain genetic profiles. These genetic profiles are detected in some children with juvenile rheumatoid arthritis and are considered genetic markers for juvenile rheumatoid arthritis. However, not all children with the .

markers develop juvenile rheumatoid arthritis, and children without the markers can develop the condition

When to seek medical advice :
If your child shows signs of joint swelling, stiffness or pain or just limps for no obvious reason, take your child to your family doctor. Also, if your child has a fever of 102 F that persists for longer than two or three days, take him or her to the doctor. A fever that signals juvenile rheumatoid arthritis may come and go one or two times during a day and last a few hours each time.

If your child has received a diagnosis of juvenile rheumatoid arthritis, take him or her to your family doctor regularly to monitor the development of the disease and its treatment. Children with pauciarticular JRA should be screened for eye inflammation regularly. A child who is diagnosed before age 7 with pauciarticular arthritis should have his or her eyes checked every three months if a blood test shows your child is anti-nuclear antibody (ANA) positive. Anti-nuclear antibodies are proteins that are generally found in people who have connective tissue or autoimmune disorders, such as arthritis.

If your child is ANA negative, your doctor will recommend an eye screening schedule based on your child's risk of developing eye problems.

Symptoms :
If you're a parent or caretaker, watch for signs of juvenile rheumatoid arthritis, particularly in young children.

The main categories of JRA are :

  • Pauciarticular JRA. This affects four or fewer joints — typically larger joints such as the knees. This is the most common form of JRA.
  • Polyarticular JRA. This affects five or more joints — typically small joints, such as those in the hands and feet. Polyarticular JRA often affects the same joint on both sides of a child's body.
  • Systemic JRA. Also known as Still's disease, systemic JRA affects many areas of the body, including joints and internal organs. This is the least common form of JRA.

Signs and symptoms of juvenile rheumatoid arthritis may include :

  • Joint swelling, with pain and stiffness. This may be more pronounced in the morning or after a nap. Commonly it affects the knees and the joints in the hands and feet. Children may complain of pain, or you might notice them limping.
  • Fever and rash. These can be associated with many medical conditions, but if they are persistent, they may signal systemic JRA. Fever and rash caused by systemic JRA may appear and disappear quickly.
  • Swelling of lymph nodes. This sign may occur in children with systemic JRA.
  • Eye inflammation. This problem, which occurs mostly in children with pauciarticular JRA, produces no signs or symptoms in most of those affected. Routine eye examinations are recommended because eye inflammation may result in blindness.

Like other forms of arthritis, JRA is characterized by times when symptoms are present (flares) and times when symptoms disappear (remissions).

Diagnosis :
If your child's pediatrician or family doctor suspects that your child has juvenile rheumatoid arthritis, he or she will refer you to a doctor who specializes in arthritis (rheumatologist) to confirm the diagnosis and for treatment.

The diagnosis of juvenile rheumatoid arthritis usually begins with a medical history and a physical examination. Diagnostic tests may include :

  • Blood tests. These may include an erythrocyte sedimentation (sed) rate test. Sedimentation rate is the speed at which your red blood cells settle to the bottom of a tube. An elevated rate can indicate inflammation. Measuring the sed rate may be used to rule out other conditions, to help classify the type of juvenile rheumatoid arthritis and to determine the degree of inflammation. Another blood test looks for antibodies in your child's blood. Whether your child has anti-nuclear antibody and rheumatoid factor in his or her blood can help the doctor to determine the type of arthritis. Anti-nuclear antibodies are proteins commonly produced by the immune systems of people with certain autoimmune diseases, including arthritis. Rheumatoid factor is an antibody commonly found in the blood of people with rheumatoid arthritis. In many children with JRA, no significant abnormality will be found in these blood tests.
  • Imaging. X-rays may be taken to exclude other conditions, such as fractures, tumors, infection and congenital defects. X-rays may also be used from time to time after the diagnosis to monitor bone development and to detect joint damage.
  • Joint fluid removal. The doctor may remove some fluid from your child's swollen joint. This can relieve pain and can help the doctor to identify the cause of the arthritis.

Complications :
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 inflammation. Eye diseases such as iritis (inflammation of the iris) and uveitis (inflammation of the uvea) may occur in children with juvenile rheumatoid arthritis, especially children with pauciarticular arthritis. These eye conditions may occur without symptoms, so it's important for children with juvenile rheumatoid arthritis to be examined regularly by an ophthalmologist. Work with your doctor to determine the best eye screening schedule for your child. Untreated eye inflammation can result in cataracts, calcium deposition in the cornea, glaucoma and, ultimately, blindness.
  • Growth interference. Juvenile rheumatoid arthritis can interfere with the development of your child's bones and growth. Some medications used to treat JRA, mainly corticosteroids, also can inhibit growth.
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.

For some children pain relievers may be the only medication needed. Others may need help from medications designed to limit the progression of the disease. Typical medications used include :

  • Nonsteroidal anti-inflammatory drugs (NSAIDs). These medications, such as aspirin, ibuprofen (Advil, Motrin, others) and naproxen (Aleve, Naprosyn), reduce pain and swelling. Because children can develop side effects such as bleeding and liver and stomach problems, be sure to use these medications under a doctor's supervision.
  • Celecoxib (Celebrex). This drug is part of a class of NSAIDs known as COX-2 inhibitors that are believed to be gentler on the stomach. The Food and Drug Administration approved celecoxib for children age 2 and older with juvenile rheumatoid arthritis in late 2006. Celecoxib hasn't been studied in children younger than 2 and in those with the systemic form of the disease. Side effects may include cough, cold, upper respiratory tract infection, abdominal pain, headache, fever, nausea, diarrhea and vomiting. COX-2 inhibitors have been found to increase the risk of heart problems in adults. Studies are being conducted to determine whether celecoxib increases heart risks in children.
  • 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 (Rheumatrex) and sulfasalazine (Azulfidine). Side effects of methotrexate may include nausea, mouth sores and liver problems. Methotrexate also may lower the number of white blood cells in your blood, leading to an increased risk of infection. Side effects of sulfasalazine may include gastrointestinal problems, such as nausea, vomiting and diarrhea, as well as headache and sore throat.
  • Tumor necrosis factor (TNF) blockers. These biologic response modifiers block an immune system protein called tumor necrosis factor, which acts as an inflammatory agent in some types of arthritis. By targeting this protein, TNF blockers can help reduce pain, morning stiffness and swollen joints. Two TNF blockers used for treating juvenile rheumatoid arthritis are etanercept (Enbrel) and infliximab (Remicade). Some people experience side effects during or shortly after these drugs are injected, including chest pain, dizziness and difficulty breathing, as well as redness, itching and swelling at the injection site. Additional side effects of biologic response modifiers may include abdominal pain, headache, respiratory infections such as tuberculosis, and other infections. These medications also may increase your risk of demyelinating disorders, conditions that damage the protective covering (myelin sheath) that surrounds nerves in your brain and spinal cord.
  • Corticosteroids. These prescription medications are for children with more severe juvenile rheumatoid arthritis. They're used to control symptoms until a DMARD takes effect or to prevent complications such as inflammation of the sac around the heart (pericarditis). Corticosteroids, such as prednisone, may be administered by mouth or by injection. But they can interfere with normal growth and increase susceptibility to infection, and generally should be used for the shortest possible duration. Stopping long-term use of corticosteroids suddenly can be dangerous, so it's important to follow a doctor's instructions on usage.

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 sports equipment for your child. A therapist may also recommend that your child make use of special supports or splints to help protect joints and keep them in a good functional position.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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