Juvenile Idiopathic Arthritis
(Juvenile Rheumatoid Arthritis)
What Is Juvenile Idiopathic Arthritis (JIA or JRA)?
Rheumatoid arthritis is a chronic (long-standing) disease that damages and eventually destroys the joints of the body. The damage is caused by inflammation, a natural response of the body's immune system that is misdirected in rheumatoid diseases. 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 idiopathic arthritis, or juvenile arthritis, is not a single disease but a group of diseases. What they all have in common is chronic joint inflammation that initially affected a child prior to 16 years of age. Besides these common features, the juvenile arthritis diseases are very different in their symptoms, their treatments, and their outcomes. The term juvenile idiopathic arthritis encompasses the five main forms of childhood arthritis: pauciarticular, polyarticular, systemic, enthesitis-related, and psoriatic arthritis. Juvenile idiopathic arthritis was formerly referred to as juvenile rheumatoid arthritis, or JRA. Juvenile idiopathic arthritis is often abbreviated JIA. Here is an outline of the five forms of JIA:
- Pauciarticular disease affects only a few joints, fewer than five. The large joints, such as the shoulder, elbow, hip, and knee, are most likely to be affected. This type of JIA is most common in children younger than 8 years of age. Children who develop this disease have a 20%-30% chance of developing inflammatory eye problems that can be serious, and these children require frequent eye examinations. Children who develop this disease when older than 8 years of age have a higher-than-normal risk of developing an adult form of arthritis. Children can outgrow the arthritis. This is the most common form of JIA.
- Polyarticular disease affects five 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.
- Systemic disease affects many systems throughout the body. Children may have high fevers, skin rash, 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.
- Enthesitis-related disease involves inflammation of the ligaments and tendons at their attachment points to adjacent bone. Additionally, the spine is typically involved with inflammation. Because of the inflammation of the spine, this form of JIA is often referred to as a spondyloarthropathy.
- Psoriatic arthritis disease is characterized by not only joint inflammation but inflammatory skin disease called psoriasis. Psoriatic arthritis features patches of inflamed scaly skin, pitting and lifting of fingernails and toenails as well as inflamed, swollen digits. There may be a history of psoriasis in other family members.
Children with JIA may experience complications specific to their type of JIA.
- The most common complications in children with JIA 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 JIA have emotional or psychological problems. Bouts of depression and problems functioning in school are the most common.
- The death rate in children with JIA is somewhat higher than in healthy children. The highest death rate in children with JIA occurs among patients with systemic JIA who develop systemic symptoms (such as pleural and pericardial disease). JIA can also evolve into other diseases, such as systemic lupus erythematosus (SLE) or scleroderma, which have higher death rates than pauciarticular or polyarticular JIA.
Treatment for JIA, similar to that for adult-type rheumatoid arthritis, has improved dramatically in the last 30 years, thanks mainly to the development of new medications.
Last Reviewed 11/17/2017
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