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Arthritis:
General Concepts > Juvenile Rheumatoid
Arthritis
What Is Juvenile Rheumatoid Arthritis?
Arthritis is a chronic condition that involves inflammation
of the joints and the connective tissue around the joints.
Juvenile rheumatoid arthritis (JRA) is a kind of arthritis
that occurs in children before age 16. It is sometimes called
juvenile chronic polyarthritis or Still’s disease. Mostly,
it involves an inflammation of the connective tissue.
With rheumatoid arthritis, the body's immune system to produces
a chemical that attacks and destroys the protective cartilage
covering the joint surface. The normally smooth and shiny joint
lining, known as synovium, becomes inflamed and swollen. Over
time, this lining thickens and the affected joints begin to
disintegrate. As the disease progresses, the bones of the joints
may move out of their correct positions.
The arthritis associated with JRA can come on quickly, or it
can develop slowly over a period of time. The disease can affect
a large number of joints in the body, or it may be confined
to only one or two joints, such as to the ankle and knee. The
number of joints involved is one way doctors classify the kind
of JRA a child has.
While JRA is seldom life threatening, it is a complicated disease,
and its effects are difficult to predict. The most common problem
for patients and their families is learning how to cope with
long bouts of a chronic condition that can be very painful
and cause deformities. There is often marked improvement, however,
or even total remission of the disease when the child reaches
puberty. And three out of every four patients with the disease
eventually enter remission with very little damage that lasts.
JRA can lead to serious complications, including a decrease
or loss of vision and total joint destruction of the major
weight-bearing joints.
In addition to its effect on the body’s joints, JRA can
affect a number of other systems in the body. It can, for instance,
affect the heart and the lungs and the tissue around those
organs. It can also have an effect on the eyes or on the skin,
and it can cause fevers, a rash, and an enlarged spleen. When
the disease is in its active state, it may affect the child’s
growth.
Causes of Juvenile Rheumatoid Arthritis?
JRA is an autoimmune disorder, in which the body mistakenly
identifies some of its own cells and tissues as foreign. The
immune system, which normally fights off bacteria or viruses,
begins to attack healthy cells and tissue instead.
Researchers suspect that the development of JRA is a two-step
process. First, something in a child's genetic makeup gives
them a tendency to develop the disease. Then an environmental
factor, such as a virus or traumatic injury, triggers the onset
of illness.
Common risk factors include:
- a family history of JRA, appearing in several generations or
in children who are siblings
- an infection such as the flu, Lyme disease, infectious mononucleosis,
or a recent immunization for rubella or DPT (diptheria-pertussis-
tetanus)
- stress, such as a divorce, separation, death in the family,
or adoption
- a severe injury or physical trauma
JRA does seem to affect girls four times more often than boys,
and it is most likely to occur at two different stages in a
child’s life — between the ages of 2 and 5 and
the ages of 9 and 12.
Symptoms of Juvenile Rheumatoid Arthritis?
Like adult onset rheumatoid arthritis, JRA involves inflammation
of the connective tissue. Although many young children with
JRA do not complain as much of joint pain, the disease often
causes redness, swelling, warmth, soreness, and the loss of
motion in the joints. The most common symptom is the persistent
swelling of particular joints accompanied by pain and stiffness
that is worse in the morning or after a nap.
JRA may flare up quickly or develop slowly over time. Sometimes
a large number of joints are involved, or as few as only one
or two, such as the ankle and knee joints. One of the earliest
signs of JRA may be limping in the morning because of an affected
knee.
The main difference between juvenile and adult rheumatoid arthritis
is that the majority of children with JRA outgrow the illness,
while adults are likely to have lifelong symptoms. Another
difference is that about 70 to 80 percent of all adults with
rheumatoid arthritis test positive for rheumatoid factor (RF)
in their blood, whereas less than half of all children with
rheumatoid arthritis are RF positive. Testing positive for
RF indicates an increased chance that JRA will continue into
adulthood.
JRA may also affect a number of other systems in the body besides
the joints. It can affect the heart and (more rarely) the lungs,
as well as the tissue around those organs. Sometimes JRA results
in damage to the eyes. It may cause fevers, rash, or an enlarged
spleen. Because JRA develops before the skeleton is mature,
JRA may affect bone development as well as the child's growth.
While JRA is seldom life threatening, it is a complex disease
requiring careful management. It is almost always unpredictable
and disruptive. The challenge for patients and their families
is learning how to cope with the pain and difficulty of long
bouts of a chronic condition during the childhood years.
General symptoms:
- joint stiffness upon waking in the morning
- limited range of motion
- slow rate of growth
- hot, swollen, painful joints
- fever, either low grade or high spiking with chills, depending
on the form of the disease
- rheumatoid rash
- rheumatoid nodules (lumps on the skin at sites of pressure)
Symptoms that indicate eye involvement:
- red eyes
- eye pain
- photophobia
- visual changes
Other symptoms:
- chest pain
- shortness of breath
- abdominal pain
Different Types of Juvenile Rheumatoid Arthritis?
Doctors classify JRA according to the number of joints involved
and the presence or absence of certain antibodies in the blood.
These antibodies are special proteins made by the immune system.
Three categories distinguish among forms of JRA, each of which
has unique features and progresses somewhat differently:
- Pauciarticular (paw-see-are-tick-you-lar) JRA involves four
or fewer joints. The most common form of JRA, affecting about
half of all children with the disease, pauciarticular JRA is
most common among girls under the age of 8. It typically affects
large joints, such as the knees.
- About 20 to 30 percent of children with pauciarticular JRA
develop problems with their eyesight. Up to 80 percent of those
with eye disease also test positive for antinuclear antibodies
or ANAs. Both ANA and JRA tend to develop at a very early age
in these children.
- Children with blood tests indicating ANA should be examined
regularly by an eye doctor (an ophthalmologist) in order to
treat or prevent serious problems such as iritis, the inflammation
of the iris, or uveitis, inflammation of the inner eye. Although
most children with pauciarticular JRA outgrow arthritis during
adolescence, some continue to have problems with their eyes
or recurring symptoms of joint pain.
-
Polyarticular JRA affects about 30 percent of all children
with the disease. In polyarticular JRA, five or more joints
are affected. The small joints of the hands and feet are most
commonly involved, usually in a symmetrical fashion. Some children
have enlarged lymph nodes (under the arms, for example) or
develop lumps under the skin known as subcutaneous nodules.
These painless lumps are usually located around the elbows,
wrists, forearms, ankles, or feet, attached to the coverings
of bones or other tissue.
- Some children with polyarticular disease have a special kind
of antibody in their blood called IgM rheumatoid factor, also
known as RF. These children usually have a more severe form
of the disease, which doctors consider to be the equivalent
of adult rheumatoid arthritis.
-
Systemic JRA is the most alarming and aggressive form of the
disease, affecting 20 percent of all children with JRA. This
is the most likely form of JRA to affect boys. Besides causing
the joints to swell, systemic JRA is characterized by intermittent
or spiking fever. Another indication is a salmon-colored smooth
rash with clearly defined irregular borders. This rash, seen
most frequently on the face and trunk, often moves around and
may appear and disappear within minutes.
- Many children who develop systemic JRA are hospitalized during
the early stages of the disease. During hospitalization many
tests are run in order to arrive at a diagnosis. Blood tests
on almost all children with systemic JRA are negative for both
RF and ANA.
- Sometimes known as Still's Disease, systemic JRA may also affect
internal organs such as the heart, liver, spleen, and lymph
nodes. Sometimes a temporary condition known as pericarditis
develops, in which there is fluid inside the sac around the
heart.
- A small percentage of children with systemic JRA develop arthritis
in many joints and have severe arthritis that continues into
adulthood.
Treatment of Juvenile Rheumatoid Arthritis?
There are two main goals for treatment:
1. to preserve mobility and joint function
2. to provide support through a long chronic illness
A doctor diagnoses JRA by carefully examining the child, considering
his or her medical history, and reading the results of laboratory
tests that help rule out other conditions. X rays are needed
if the doctor suspects injury to the bone or unusual bone development.
Symptoms must be present for at least 6 weeks for the doctor
to consider a diagnosis of JRA.
JRA is best managed using a team approach. A pediatrician,
family physician, or other primary care doctor usually manages
treatment, with the help of other doctors. These may include
pediatric rheumatologists (doctors specializing in childhood
arthritis), ophthalmologists (eye doctors), orthopaedic surgeons
(bone specialists), and physiatrists (rehabilitation specialists),
as well as physical and occupational therapists. Sometimes
counseling is used to help the child and family cope with the
emotional aspects of the illness.
The goals of treatment are to preserve a high level of physical
and social functioning, allowing the child to maintain a good
quality of life despite the illness. Treatment aims to reduce
pain and swelling, maintain full movement in the affected joints,
and identify, treat, and prevent complications. Medication
and physical therapy are used to achieve these goals.
- Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) such as aspirin,
ibuprofen (Motrin, Advil, Nuprin) and naproxen or naproxen
sodium (Naprosyn, Aleve) are often are the first type of medication
used.
- Most doctors hesitate to treat children with aspirin because
of the possibility that it will cause bleeding problems, upset
stomach, liver problems, or Reye's syndrome. But for some children
aspirin in the correct dose, as measured by blood test, can
effectively control JRA with few serious side effects.
-
Disease-Modifying Anti-Rheumatic Drugs (DMARDs) are often used
if NSAIDs do not adequately relieve symptoms. Because DMARDs
may take weeks or months to slow the progress of JRA, they
are often taken in combination with a NSAID.
- Various types of DMARDs are available, but today methotrexate
is most commonly used. Because only small doses of methotrexate
are needed to relieve arthritis symptoms, potentially dangerous
side effects rarely occur. The most serious complication is
liver damage, but it can be avoided with regular blood tests
and careful monitoring.
-
Corticosteroids, given either orally or intravenously, may
be needed to treat serious symptoms such as inflammation of
the sac around the heart (pericarditis).
Because corticosteroids can interfere with a child's normal
growth and cause other side effects, such as a round face,
weakened bones, and increased susceptibility to infections,
their use must be carefully monitored. It is important to follow
the doctor’s instructions about how to take or reduce
the dosage of corticosteroids, as it can be dangerous to stop
taking these medications suddenly.
Physical therapy is another vital part of a child's treatment
plan. Besides helping to maintain muscle tone, exercise can
preserve and recover the range of motion of the joints. It
also helps a child maintain self-confidence and emotional strength
to cope with the ups and downs of JRA.
A physical therapist helps design an appropriate exercise program
for a person with JRA, and may recommend using splints and
other devices to help the joints grow evenly. Swimming is highly
recommended because it uses many joints and muscles without
putting weight on the body. Whirlpool treatment and the use
of heat or ice is also beneficial in reducing discomfort.
Surgery may occasionally be recommended for children with JRA,
when there are obvious deformities of the joints. An orthopedic
surgeon can advise the patient and his or her family about
the kinds of surgery that may be appropriate in helping to
control the disease.
Living with Juvenile Rheumatoid Arthritis?
When a child has JRA, the entire family must learn to deal
with special challenges. A child with JRA may find it difficult
to participate in social and after-school activities, as well
as the requirements of schoolwork. It helps to work closely
with the school to develop suitable lesson plans for the child,
as well as to educate the child’s teacher and classmates
about JRA. Children with JRA may need to be educated at home
periodically, and they may benefit from tutoring or simple
adjustments in the school routine.
It is helpful if family members and teachers treat the child
as normally as possible, making adjustments as needed during
the course of the disease. When symptoms are under control,
a child can be more active, even participating in sports. At
other times, activities need to be limited.
It is important to ensure that the child receives appropriate
medical care, tailored to the specific needs of the child.
What works for one child may not work for another. If the medications
that the doctor prescribes do not relieve symptoms or if they
cause unpleasant side effects, patients and parents should
discuss other choices with their medical team.
The American Juvenile Arthritis Organization runs support groups
for the families of people with JRA. Support groups provide
the chance to network with other young people and parents of
children with JRA. The support offered by other families is
often invaluable in helping a family deal with the challenges
of living with juvenile rheumatoid arthritis.
The information
provided herein is not intended to be a substitute for professional
medical advice. You should not use this information to diagnose
or treat a health problem or disease without consulting a licensed
physician.
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