Juvenile Rheumatoid Arthritis (JRA)

Juvenile Rheumatoid Arthritis (JRA)

Article
Bone, Muscle, & Joint
Diseases & Conditions
+1
Contributed byKrish Tangella MD, MBAOct 18, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • JIA (Juvenile Idiopathic Arthritis)
  • JRA (Juvenile Rheumatoid Arthritis)
  • Juvenile Chronic Arthritis

What is Juvenile Rheumatoid Arthritis? (Definition/Background Information)

  • Juvenile Rheumatoid Arthritis (JRA) is a chronic autoimmune systemic inflammatory disorder that affects the lining of joints, surrounding tissues, and organs, within the body (chiefly the hands and feet), of children and adolescents.
  • Unlike the degeneration of cartilage associated with osteoarthritis, Juvenile Rheumatoid Arthritis affects the lining of the joint. This causes excessive pain and swelling, resulting in bone erosion and joint deformity.
  • Children, who develop JRA often experience fatigue, stiffness, and some joint pain.

There are seven types of Juvenile Rheumatoid Arthritis:

  • Systemic Juvenile Idiopathic Arthritis: This type of arthritis affects the child’s entire body. Symptoms often include abnormally high fevers, and the spleen and lymph nodes become enlarged. Eventually, many joints are affected, causing pain, swelling, and stiffness
  • Oligoarthritis: This type of arthritis affects four or fewer joints, with the most common joint affected being the knee and wrist. The two different types of oligoarthritis include persistent and extended, which are determined by the number of joints that are affected
  • Polyarticular Arthritis, Rheumatoid Factor Negative: Is more prevalent in girls than boys and affects five or more joints. The most common joints affected are the small joints within the hand, or weight-bearing joints, such as the knees, hips, ankles, feet, and neck
  • Polyarticular Arthritis, Rheumatoid Factor Positive: This type of arthritis affects only a small percentage of children (approximately 15%, which is about 3% of all children with Juvenile Rheumatoid Arthritis). JRA resembles adult Rheumatoid Arthritis, and children are more likely to experience joint damage with erosions, with this type of JRA, than with the other types
  • Psoriatic Arthritis: Children associated with this type of JRA, show the presence of ‘psoriasis rash’, and may have an immediate family with psoriasis. Fingernails and/or toenails may also be affected by the disorder
  • Enthesitis-Related Arthritis: Enthesitis-Related Arthritis mostly affects the lower extremities and the spine. Children may experience inflammation where tendons attach to the bones, such as where the Achilles tendon attaches to the back of the heel. This JRA type is also included in a specialized group, called juvenile ankylosing spondylitis (where joints within the lower back are inflamed) and arthritis associated with inflammatory bowel disease (Crohn's disease and ulcerative colitis)
  • Undifferentiated Arthritis: This type of arthritis does not seem to be associated with any of the other types of arthritis categories, or it may fit into multiple categories. The initial signs of arthritis, which can be subtle or noticeable, may include walking difficulties, tender wrist, finger, or knee. Swelling within the joints may occur suddenly, and the joints may remain swollen. Stiffness of the neck, hips, or other joints can also occur. There may be rashes at multiple locations that appear and disappear spontaneously/suddenly. Abnormally high fevers that tend to increase in the evenings and unexpectedly disappear; characteristics that are similar to Systemic Juvenile Idiopathic Arthritis

Who gets Juvenile Rheumatoid Arthritis? (Age and Sex Distribution)

  • Children or adolescents (16 years old and younger) of any race/ethnic groups, and gender can develop Juvenile Rheumatoid Arthritis, which is the most common type of arthritis that develop in juveniles
  • The condition is mostly seen in children between the ages of 7-12 years, though it may affect infants and older children too
  • Girls are more likely to develop this disorder, than boys

What are the Risk Factors for Juvenile Rheumatoid Arthritis? (Predisposing Factors)

  • Girls are at an increased risk of developing Juvenile Rheumatoid Arthritis than boys
  • Studies have indicated that Rheumatoid Arthritis (RA) is more common in individuals with a certain genetic make-up. Individuals having an immediate family member/relative with a history of RA, have an increased risk of developing the disorder

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Juvenile Rheumatoid Arthritis? (Etiology)

  • Juvenile Rheumatoid Arthritis occurs when the body immune system attacks the synovial membranes that surround the joints. The resulting inflammation that occurs due to an abnormal immune response, causes thickening of the synovial membrane (joint linings)
  • Prolonged inflammation may cause damage to the cartilages and bones of the joint. Due to this, the surrounding tendons and ligaments that hold the joint in place are weakened and stretched, slowly causing the joint to lose its alignment and structure
  • Some individuals are more prone to infections than others. Research has shown that this may be due to the presence of certain type of genes in them. Infections with certain viruses and bacteria may trigger the development of Rheumatoid Arthritis
  • Hence, it has been suggested, that individuals with such genes may have a higher risk of developing RA. Research is currently being undertaken to understand better, the genetic risks involved in this autoimmune disorder

What are the Signs and Symptoms of Juvenile Rheumatoid Arthritis?

Signs and symptoms of Juvenile Rheumatoid Arthritis, which generally affect multiple joints, include:

  • Noticeable swelling and tenderness of the joints, decreased range of motion
  • Feeling of weakness (inactive children), loss of weight, ‘feeling sick’
  • Children tend to limp (first indicators of this condition)
  • Poor appetite
  • Prolonged stiffness of the joints, especially observed in the mornings (that may improve during the day, due to use of the limbs)
  • Firm lumps beneath the skin, swollen lymph nodes
  • Elevated body temperatures, rashes
  • On progression of the condition joint deformity with joint contracture (bent joints) and damage is observed
  • Common locations of RA manifestation include small joints of the feet, hands, especially the wrists, ankles, and knees. Other locations may include the spine, jaw, hip, and shoulder

How is Juvenile Rheumatoid Arthritis Diagnosed?

Early-stage diagnosis of Juvenile Rheumatoid Arthritis can sometimes be difficult, often due to the symptoms resembling other conditions. There are a variety of diagnostic tests a physician may use to diagnose JRA, which include:

  • Physical examination: The physician will perform a thorough physical examination to determine, if the individual has JRA. In addition to this, a complete medical history may aid in arriving at a definitive diagnosis. The physician will also check the joints for swelling, redness, tenderness, its reflexes and muscle strength
  • Erythrocyte sedimentation rate (ESR): ESR is a blood test that can help the healthcare provider identify any inflammatory activity within the body and arrive at a definitive cause for Juvenile Rheumatoid Arthritis. During this test, a blood sample is drawn from an artery or vein using a syringe, which is then taken to a laboratory for analysis
  • C-reactive protein (CRP) test: This blood test measures the amount of a protein, called C-reactive protein, in blood. It also provides information on the inflammation condition of the body
  • Anti-nuclear antibody (ANA) test: ANA test identifies the antinuclear antibodies within the blood. Antinuclear antibodies are proteins commonly produced by the immune systems of children, during specific autoimmune disease conditions, such as JRA
  • Rheumatoid factor (RF) test: A RF test measures the amount of rheumatoid factor in blood
  • Cyclic citrullinated peptide (CCP): Similar to rheumatoid factor, the CCP is another type of antibody that is commonly found within the blood of children with JRA
  • X-rays: X-rays use radiation in order to produce images of the joints. This can help the physician rule out other possible conditions, such as fractures, tumors, infection, or congenital defects
  • Other imaging tests include: MRI scan, ultrasound
  • Blood test: During the blood test, a needle is used to obtain blood samples from an artery or vein, which are then sent to a laboratory for analysis
  • Joint fluid analysis: Occasionally, fluid may accumulate around a joint, resulting in pain and disability. Analysis of such joints may provide evidence regarding the cause of joint fluid accumulation. A needle is inserted into the joint space, and the fluid aspirated with a syringe. The aspirated fluid is sent for a laboratory analysis; the tests a laboratory may perform include:
    • Analyzing what cells are present in the joint fluid
    • Chemical composition of the fluid
    • Examinations of the fluid under a microscope for the presence of crystals
    • If an infection is suspected as the cause for fluid accumulation, then a joint fluid culture may be performed, to remove fluid from the joint

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Juvenile Rheumatoid Arthritis?

Complications of Juvenile Rheumatoid Arthritis could include:

  • Inflammation of the eye (uveitis), which is one of the most noteworthy complication associated with JRA
  • Growth issues, abnormal bone development problems

How is Juvenile Rheumatoid Arthritis Treated?

Currently, the exact underlying cause of Juvenile Rheumatoid Arthritis is unknown. Although, conservative treatments may be used to help to reduce pain and improve range of motion within the joint. These include:

  • Use of specific assistive devices, such as a cane or walker is recommended. This may help individuals perform some of their basic everyday activities
  • Immunosuppressant drugs may be prescribed to control an active immune system
  • Tumor necrosis factor-alpha (TNF-alpha) drugs may be used to block the proteins, responsible for inflammation. These medications can help reduce pain, stiffness in the morning, tenderness or swelling within the joints
  • Several other drugs that may be used to treat RA include anakinra, abatacept, rituximab, and tocilizumab
  • Disease-modifying anti-rheumatic drugs (DMARDs) may be administered to decrease the magnitude of joint damage that could occur
  • Any physical therapy exercises that could aid in strengthening and improving the flexibility of the joints, and also help reduce discomfort. These are generally used, only after the signs and symptoms have been controlled or decreased
  • Non-steroidal anti-inflammatory oral medications, such as ibuprofen and naproxen, may be used to treat Rheumatoid Arthritis. These medications may help decrease the pain and joint swelling
  • Corticosteroid injections help provide temporary relief of symptoms, and in improving the range of motion. It is important to note that corticosteroid injections only give temporary relief. Prolonged episodes of such injections, may injure the joints in the long-run
  • Surgical treatment is rarely used in children with Juvenile Rheumatoid Arthritis. However, surgical interventions may be recommended, if excessive pain and joint damage are present

How can Juvenile Rheumatoid Arthritis be Prevented?

  • Currently, there are no preventable measures associated with Juvenile Rheumatoid Arthritis
  • If there is an early, aggressive, and proper treatment of this inflammatory disorder, then the progression of this disabling condition, could be slowed down or decreased

What is the Prognosis of Juvenile Rheumatoid Arthritis? (Outcomes/Resolutions)

  • The long-term prognosis of Juvenile Rheumatoid Arthritis depends on the severity of this inflammatory disorder
  • If during the early stages, proper and aggressive treatment of JRA is undertaken, then in some patients, the condition may show an improvement, without giving rise to any serious complications
  • Other factors that determine the course of outcome of the condition include,if this disorder is in a flared-up state, in an arthritic remission period, or whether it was correctly controlled with treatment
  • A high percentage of individuals, who develop JRA, may experience a lifetime of gradual inflammation, associated with this debilitating disorder

Additional and Relevant Useful Information for Juvenile Rheumatoid Arthritis:

Rheumatoid Arthritis is an auto-immune inflammatory disorder that predominantly affects the hands and feet, of middle-aged to elderly adults (typically in the 40-60 year age group).

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Krish Tangella MD, MBA

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