Juvenile Ankylosing Spondylitis

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Juvenile Ankylosing Spondylitis (JAS) is a type of arthritis that affects the spine and the sites where the muscles, tendons, and ligaments are attached to bone. The disease causes inflammation of the spine and large joints, resulting in stiffness and pain. The disease may result in erosion at the joint between the spine and the hip bone, and the formation of bony bridges between vertebrae in the spine, fusing those bones. In addition, bones in the chest may fuse.

JAS is considered to be a multifactorial condition. Multifactorial inheritance means that "many factors" are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender is affected more frequently than the other in multifactorial traits. Multifactorial traits do recur in families because they are partly caused by genes.

A group of genes on chromosome 6 code for HLA antigens play a major role in susceptibility and resistance to disease. Specific HLA antigens influence the development of many common disorders. Some of these disorders, such as JAS, are autoimmune related and inherited in a multifactorial manner. When a child has a specific HLA antigen type associated with the disease, he/she is thought to have an increased chance to develop the disorder. The HLA antigen associated with JAS is called B27. Children with the B27 HLA antigen are thought to have an increased chance to develop JAS; however, it is important to understand that a child without this antigen may also develop juvenile ankylosing spondylitis. This means HLA antigen testing is not diagnostic or accurate for prediction of the condition. Males are affected with JAS three times more often than females.

JAS is one of four disorders which are classified as spondyloarthropathies. The other disorders are psoriatic arthritis, inflammatory bowel disease, and Reiter's syndrome. These disorders have similar features such as:

  • inflammation of the spine and sacroiliac joints
  • family history of the disease
  • similar non-arthritis symptoms
  • absence of rheumatoid factor in the blood

  • Symptoms:
    Symptoms of JAS tend to occur and disappear over periods of time. The following are the most common symptoms of JAS. However, each child may experience symptoms differently. Symptoms may include:

  • back pain, usually most severe at night during rest
  • early morning stiffness
  • bending forward tends to relieve the pain
  • inability to take a deep breath, if the joints between the ribs and spine are affected
  • appetite loss
  • weight loss
  • fatigue
  • fever
  • anemia
  • pain at the site of attachment of muscles, ligaments, and/or tendons to bone
  • joint pain, particularly in the legs
  • vague pain, usually in the buttocks, thighs, heels, or near the shoulders
  • eye inflammation that is painful and causes redness and light sensitivity; may have frequent recurrences of eye inflammation
  • organ damage, such as the heart, lungs, and eyes

  • Symptoms of this disease may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.

    The specific cause of juvenile ankylosing spondylitis is unknown, but genetic factors seem to play a role.

  • About 95% of people who have juvenile ankylosing spondylitis also have a genetic marker known as human leukocyte antigen-B27 (HLA-B27).
  • About eight people in 100 among American Caucasians are born with the HLA-B27 gene. The gene is much less common among African Americans.

  • Genetic markers are protein molecules found on the surfaces of cells. The HLA markers enable the body's immune system to distinguish between "self" and "other."

    Juvenile ankylosing spondylitis may be triggered by certain types of bacterial or viral infections that activate an immune response that does not shut off after the infection is healed. The immune system then attacks the body's own tissue. A disorder caused by the body's own immune system is called an autoimmune disease.

    . . . Is Ankylosing Spondylitis Inherited?
    If one parent has HLA-B27 and ankylosing spondylitis, there clearly is some increased risk that the B27 gene and disease will be passed on to a child. However, only about 2% of people with HLA-B27 develop ankylosing spondylitis.

    Ankylosing spondylitis occurs more frequently in some ethnic groups. In the United States, it occurs most frequently among Native Americans and is almost never found among African Americans. Chinese individuals have the gene much more often than Japanese individuals.

    The goal of treatment for JAS is to reduce pain and stiffness, prevent deformities, and help your child maintain as normal and active a lifestyle as possible.

    Treatment may include:

  • nonsteroidal anti-inflammatory medication, NSAIDs, to reduce pain and inflammation
  • short-term use of corticosteroids to reduce inflammation
  • maintain proper posture
  • regular exercise, including exercises that strengthen back muscles
  • physical therapy

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