Juvenile Ankylosing
Spondylitis
What is juvenile ankylosing spondylitis?
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. "Ankylosing" means stiff or rigid, "spondyl" means
spine, and "itis" refers to inflammation. 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 (the sacroiliac joint), 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
(either males or females) 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 (or "genetic susceptibility") to develop JAS; however, it is
important to understand that a child without this antigen may also develop
JAS. 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 (RF) in the blood (an antibody found in
the blood of most, but not all, people who have rheumatoid arthritis, as
well as other rheumatic diseases)
Who is affected by juvenile ankylosing spondylitis?
JAS is more common among adolescent males. The disease affects approximately
one in
100 Caucasian boys. The average age at onset of the disease is 10 years or
older.
What are the symptoms of juvenile ankylosing
spondylitis?
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
- stooped posture in response to back pain (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 (enthesitis)
- 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 juvenile ankylosing spondylitis may resemble other medical
conditions or problems. Always consult your child's physician for a
diagnosis.
How is juvenile ankylosing spondylitis diagnosed?
In addition to a complete medical history and physical examination,
diagnostic procedures for juvenile ankylosing spondylitismay include:
- x-rays - a diagnostic test which uses invisible
electromagnetic energy beams to produce images of internal tissues,
bones, and organs onto film.
- erythrocyte sedimentation rate (ESR or sed rate) - a
measurement of how quickly red blood cells fall to the bottom of a test
tube. When swelling and inflammation are present, the blood's proteins
clump together and become heavier than normal. Thus, when measured, they
fall and settle faster at the bottom of the test tube. Generally, the
faster the blood cells fall, the more severe the inflammation. (People
with JAS often have an elevated ESR.)
- detection of (HLA-B27) antigen - HLA-B27 is an antigen (a
substance that causes the body to produce antibodies to fight bacteria,
viruses, and other "foreign" particles to prevent infection) whose
presence may indicate the presence of some type of autoimmune disease (a
condition in which the body's own defenses begin to attack the body's
cells).
Treatment for juvenile ankylosing spondylitis:
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.
Specific treatment for juvenile ankylosing spondylitis will be
determined by your child's physician based on:
- your child's overall health and medical history
- extent of the condition
- your child's tolerance for specific medications, procedures, and
therapies
- expectation for the course of the disease
- your opinion or preference
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
More Information
Schneider's Children's Hospital
Division of Rheumatology
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