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Juvenile Rheumatoid Arthritis (JRA)
Wednesday, 16 July 2003
Sunday, 03 December 2006

What

Juvenile rheumatoid arthritis (JRA), which is different from adult rheumatoid arthritis, is a condition that begins in children less than 16 years old and involves irritation and inflammation of the body’s joints. The inflammation in JRA is chronic, so it lasts for a prolonged period of time. JRA is considered an autoimmune disease, which means that the body’s immune system, which normally attacks harmful or unknown substances, begins attacking the child’s normal tissue. In JRA, the tissues affected are specifically the joints and connective tissue but may also be located in the internal organs. Juvenile rheumatoid arthritis is divided into three different groups, depending upon the symptoms the child has during the first six months of the illness. • The first group is known as Pauciarticular (“few joints”) JRA. This is the most common type and affects 40-50% of children with JRA. The children have 4 or fewer large joints affected. These children have an increased risk of developing inflammation in their eyes (uveitis) compared with the other forms of JRA, especially if they have a blood test positive for “antinuclear antibody” (ANA) factor. This factor is a marker of the activity against the child’s own tissues. • The second group is Polyarticular (“many joints”) JRA. This category affects 5 or more joints, but these children do not have significant symptoms affecting other parts of their body. This is the second most common type of JRA and makes up 30-40% of children with JRA. They are not as likely as Pauciarticular JRA to have uveitis. • The third group is Systemic JRA which occurs in 10-20% of children with JRA. This category is characterized by symptoms affecting the entire body, such as fever and rash. The arthritis typically presents within 6 months of the rash. This type of JRA may also affect internal organs, such as the brain, lungs, heart, liver, spleen, lymph nodes and blood cells.

Who

Juvenile rheumatoid arthritis affects 10-20 children out of every 100,000, although there is thought that even more may be affected. The symptoms of JRA must be present before the child is 16 years old. Pauciarticular JRA usually occurs in young childhood, and Polyarticular JRA can be diagnosed in younger children with a second peak in the teenage years. Systemic JRA can occur at any age prior to 16 years. Like other autoimmune diseases, JRA is more common in females although the systemic form is fairly equal between males and females.

Signs and Symptoms

Pauciarticular JRA is characterized by: • joint problems: There is arthritis (swelling of the joint with pain with movement, decreased movement, or warmth of the joint) and/or morning stiffness of 4 or fewer joints. • eye concerns: Occasionally there is eye pain or sensitivity to light, and this may occur without arthritis. Even though areas of the eye may be inflamed or irritated, there may be no obvious eye redness. Polyarticular JRA is characterized by: • joint problems: There is mild to moderate joint pain which may only present when the joint is moving, morning stiffness in the joints and decreased ability to move the affected joints. • non-joint problems: These children may also be more tired than normal, not feel well and experience weight loss. Systemic JRA is characterized by symptoms which affect areas throughout the body including: • joint/muscle problems: They have arthritis, more commonly in a pattern like Polyarticular JRA. There may also be muscle aches. • fevers: These children will often have high fevers once or twice daily which go away without medicine. They may appear more ill during the fevers. • heart/lungs: They may complain of chest pain or difficulty breathing. There can be irritation of the tissues that surround the heart and lungs. • liver/spleen: The liver and spleen may be enlarged in these patients. • lymph nodes/blood cells: There can be swelling of lymph nodes. These children may also be anemic or have abnormal blood clotting ability. • rash: The rash is typically a pink rash that comes and goes and is worse with the fevers. If the child scratches or rubs the rash, it may spread along the skin. • eyes: Uveitis is less common in these patients compared with those with Pauciarticular JRA, but a good eye exam is important. • additional symptoms: The children may feel more tired than normal, not feel well and experience weight loss.

Possible Causes

Although the exact cause of juvenile rheumatoid arthritis is not known, there are many theories. Most people believe there is a combination of factors which result in the disease. The child’s normal immune system, which is designed to attack substances which could be harmful, instead begins to attack the child’s normal tissue. This tissue may be located in the joints or in the body’s other organs, and it leads to irritation and inflammation of these tissues. Since some of the children with JRA have other family members with JRA or other types of arthritis, there may also be a genetic predisposition. Additionally there may be substances in the environment or even infections which make a child more likely to develop JRA, although these have not yet been proven.

Diagnosis

Juvenile rheumatoid arthritis cannot be diagnosed with a single test. Since many of the symptoms of JRA are also symptoms of other illnesses, it is often a diagnosis made after the other possibilities have been excluded. The history of the child’s illness and the physical exam provide clues to the doctor as to whether or not the child may have one of the three types of JRA. To diagnose JRA, the child must be less than 16 years old and there must be arthritis in at least one joint. The symptoms also have to have been present for at least 6 weeks. Some blood tests may be useful. If the antinuclear antibody test (ANA) is positive, this is a way to monitor how actively the body is attacking itself and also to determine whether the child is at risk for future eye disease. Blood counts may indicate not only anemia but also increased inflammatory activity. X-rays showing inflammation of the affected joints can be helpful in the diagnosis. However, the physical exam and the patient history are most important. Ultimately, the diagnosis is based on the overall clinical picture.

Treatment

Children can often be treated as outpatients and do not necessarily require admission to the hospital. • Since many of the symptoms are in the joints and muscles, physical therapy and a good exercise program are very important. These children should also be encouraged to participate in play, exercise and extracurricular activities, which help keep their joints moving. This should not be over-done and under the guidance of a doctor or physical therapist. • To treat the inflammation and improve joint symptoms, non-steroidal anti-inflammatory drugs (NSAIDS) like ibuprofen and naprosyn are used. Often, there is a need to use other medications to help treat the inflammation. Steroids taken by mouth, intravenously or injected into the affected joint help suppress active flares of JRA. Steroids are usually taken for short periods of time to prevent long-term side effects like weight gain, poor growth, and immune suppression if they are taken continuously. Children who have to take steroids frequently will usually start taking a second medication to help decrease the amount of steroid they need to take. This may include methotrexate, which in the past has been used in higher doses to treat cancer but in lower doses helps control the inflammation in JRA. • It is important that these children eat a diet with sufficient amounts of calcium, iron and protein. • To monitor for signs of the eye inflammation (uveitis), regular visits to the eye doctor (ophthalmologist) are necessary.

Prognosis

Complete remission occurs in 35-73% of cases, and the best outcomes occur when the disease is able to be controlled early on. Therefore, doctors are usually initially aggressive with medications in order to quiet down the inflammation. Most children tend to do well and not have symptoms in adulthood; the ones who do not have as good a response are those who develop symptoms later in childhood, those with polyarticular disease and those who have hip involvement.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

KidsHealth
This is a great site designed for kids to learn more about their own health and illnesses.

National Institute of Arthritis and Musculoskeletal and Skin Disease
A very thorough site with great information about the disease as well as links to support groups and ongoing trials.

The Arthritis Foundation
This is a comprehensive site with easy to read information about JRA as well as links to support networks.

University of Washington Orthopedics and Sports Medicine
This is an excellent site for a comprehensive explanation of JRA. The site is a little lengthy and is quite detailed. However, it is very thorough yet intelligible and parent-friendly. There is also a figure explaining joint inflammation.

Google Search for Juvenile Rheumatoid Arthritis (JRA)

References and Sources

Lehman T, “Clinical manifestation and diagnosis of polyarticular onset juvenile rheumatoid arthritis,” UpToDate. Online: www.uptodate.com. Accessed on November 12, 2006. Lehman T, “Clinical manifestation and diagnosis of systemic onset juvenile rheumatoid arthritis,” UpToDate. Online: www.uptodate.com. Accessed on November 12, 2006. Lehman T, “Management and complications of systemic onset juvenile rheumatoid arthritis,” UpToDate. Online: www.uptodate.com. Accessed on November 12, 2006. Lehman T, “Management of polyarticular onset juvenile rheumatoid arthritis,” UpToDate. Online: www.uptodate.com. Accessed on November 12, 2006. Lehman T, “Pauciarticular onset juvenile rheumatoid arthritis,” UpToDate. Online: www.uptodate.com. Accessed on November 12, 2006. Miller M, “Juvenile Rheumatoid Arthritis,” emedicine, http://www.emedicine.com/ped/topic1749.htm, Accessed November 12, 2006. Last updated June 14, 2006. Wallace CA, “Current management of juvenile idiopathic arthritis,” Best Pract Res Clin Rheumatol. 2006; 20(2):279-300.