Juvenile Idiopathic Arthritis
It may begin with a swollen knuckle, a spiking fever, or an unexplained rash. But
no matter what symptoms appear, hearing the word "arthritis" in a diagnosis for your
child can be unexpected and confusing.
Arthritis is an inflammation of the joints, meaning that the joints get swollen,
warm, and painful. Nearly 300,000 children in the United States have some sort of
arthritis. Arthritis can be short-term — lasting for just a few weeks or months,
then going away forever — or it can be chronic and last for months or years.
In about half of cases, it can last a lifetime.
The most prevalent form of juvenile arthritis is juvenile idiopathic arthritis
(JIA) (also known as juvenile rheumatoid
arthritis, or JRA). It is very different from adult
rheumatoid arthritis.
What Causes JIA?
It's not known exactly what causes JIA in kids. Research indicates that it is an
autoimmune disease. In autoimmune diseases, white blood cells can't tell the difference
between the body's own healthy cells and germs like bacteria
and viruses. The immune system, which is supposed to protect the body from these
harmful invaders, instead releases chemicals that can damage healthy tissues and cause
inflammation and pain.
To effectively manage and minimize the effects of arthritis, an early and accurate
diagnosis is essential. By understanding the symptoms and characteristics of each
type of JIA, you can help your child maintain an active, productive lifestyle.
Types of JIA
JIA usually appears in kids between 6 months and 16 years old. The first signs
often are joint pain or swelling or warm joints. Many rheumatologists (doctors specializing
in joint disorders) find that the greater the number of joints affected, the more
severe the disease and the less likely that the symptoms will eventually go into total
remission. Remission is a medical term for temporary or permanent recovery.
There are seven types of JIA:
- Systemic JIA. Affects the whole body. Symptoms include high fevers
that often increase in the evenings and then may suddenly drop to normal. During the
onset of fever, the child may feel very ill, appear pale, or develop a rash. The rash
may suddenly disappear and then quickly appear again. The spleen and lymph nodes might
become enlarged. Eventually many of the body's joints are affected by swelling, pain,
and stiffness. Rashes may suddenly appear and disappear, developing in one area and
then another. High fevers that tend to increase in the evenings and disappear are
characteristic of systemic JIA
- Oligoarthritis. Affects four or fewer joints, often the knee
or ankle. Symptoms include pain, stiffness, or swelling in the joints. The two
types of oligoarthritis, persistent and extended, are determined by how many joints
are ultimately involved.
- Polyarticular arthritis, rheumatoid factor negative.
About 1 in 4 kids and teens with JIA have polyarthritis, which affects
more girls than boys. Symptoms include swelling or pain in five or more joints. The
small joints of the hands are affected as well as the weight-bearing joints like the
knees, hips, ankles, feet, and neck. A low-grade fever also might develop, as well
as bumps or nodules in areas of the body subjected to pressure from sitting or leaning.
- Polyarticular arthritis, rheumatoid factor positive. This
type of JIA behaves the most like adult rheumatoid arthritis, and kids who have it
have a protein called rheumatoid factor (RF) or anti-cyclic citrullinated peptide
(CCP antibody) in their blood. Kids with polarticular JIA are at a higher
risk of joint damage with erosions than in the other forms of JIA.
- Psoriatic arthritis. Kids with this also have the psoriasis rash
(a scaly red rash that can start behind the ears, on the eyelids, elbows, knees, or
scalp) themselves or a close relative with psoriasis. Their fingernails and toenails
might be affected by the condition.
- Enthesitis-related arthritis. This type of arthritis often affects
the legs and spine. Kids also might have inflammation at the entheses – areas
where tendons join bones (where the Achilles tendon attaches to the back of the heel).
Enthesitis-related
arthritis includes a special group call juvenile ankylosing
spondylitis (where joints of the low back are inflamed) and arthritis associated
with inflammatory bowel disease
(Crohn's disease and ulcerative colitis).
- Undifferentiated arthritis. Arthritis that doesn't fit into any
of the above categories or fits into more than one of the categories.
The first signs of arthritis, which can be subtle or obvious, include limping
or a sore wrist, finger, or knee. Joints may suddenly swell and remain enlarged. Stiffness
in the neck, hips, or other joints also can occur.
Inflammation of the iris (the colored area of the eye) may happen with or without
active joint symptoms in any type of JIA. This inflammation, more likely to happen
in girls than boys, is called iridocyclitis, iritis, or uveitis. Kids and teens with
JIA should see an ophthalmologist (an eye doctor) regularly to check for this.
Diagnosis
To diagnose JIA, the doctor will take ask you questions about your child’s
symptoms, find out whether other family members have had similar problems, and
do a thorough physical examination. The doctor may order X-rays or blood tests to
rule out other conditions or infections, such as Lyme
disease, that may cause similar symptoms or occur along with the arthritis.
Other tests may include:
- CBC (complete blood count),
a common blood test that checks all the basic cell types in blood, including
red blood cells, white blood cells, and platelets. Knowing the amount and appearance
of each cell type in a person’s blood can help doctors identify many medical
conditions.
- Blood culture, a test
to detect bacteria that cause infections in the bloodstream.
- Bone marrow biopsy,
a test that allows doctors to look at blood where it's formed (in the bone marrow)
to look for conditions such as leukemia.
- Erythrocyte sedimentation
rate, which checks how rapidly red blood cells settle to the bottom of a test
tube. This rate often increases in people when inflammation is occurring in the body.
- C-reactive protein (CRP)
is a protein that the liver releases into the blood at the start of infection or inflammation;
this level can rise if a person is having an arthritis flare-up.
- A test for rheumatoid factor (RF) and cyclic citrullinated peptide antibody (CCP),
substances made in the blood of children with some forms of JIA. But it's found more
often in adults with rheumatoid arthritis.
- ANA (antinuclear antibody), a blood test to detect autoimmune diseases. It's also
useful in predicting which kids are likely to have eye disease with JIA.
- X-rays of the affected joints, and sometimes an MRI,
to detect changes in bone and joints to evaluate the causes of unexplained bone and
joint pain. In some cases, doctors may do a test called a bone scan.
In some cases, the doctor may want an orthopedic surgeon to examine your child's
joints and take samples of joint fluid or synovium (the lining of the joints) for
examination and testing.
Treatments
In many cases, JIA is treated with a combination of medication, physical therapy,
and exercise. In some cases, a child may require corticosteroid injections into the
joint. In very rare cases, kids and teens may need surgery. The health care providers,
including the primary care physician, rheumatologist, and physical therapist, will
work together to develop the best method of treatment.
The goals of treatment are to relieve pain and inflammation, slow down or prevent
the destruction of joints, and restore use and function of the joints to promote optimal
growth, physical activity, and social and emotional development.
Medications
For inflammation and pain, the doctor or pediatric rheumatologist may prescribe
nonsteroidal anti-inflammatory drugs (NSAIDs), like ibuprofen, such as Advil, Motrin,
or naproxen (Naprosyn or Aleve). These can help reduce inflammation and pain by limiting
the release of harmful chemicals from white blood cells.
Higher or lower dosages might be needed, depending upon your child's response to
the medication. The doctor or rheumatologist should explain what the medication is
meant to do and what side effects, if any, it could cause. It's important for your
child to continue taking the medication until the doctor says to stop.
Doctors sometimes prescribe corticosteroids (like prednisone) for arthritis flares,
but they try to keep these to a minimum to reduce problems that can come with prolonged
steroid use, such as skin changes, weight gain, abnormal blood pressure, diabetes,
and bone mass changes.
If NSAIDs don't control joint inflammation, your doctor may prescribe other medications
such as methotrexate. In addition, treatment options now include a newer class of
medications called biologics. The U.S. Food and Drug Administration (FDA) has
approved many of these medicines for kids and teens with JIA. Some are subcutaneous
injections (injections given just under the skin) that can be done at home. Others
are intravenous (IV) infusions through a vein that are done regularly at the hospital.
Physical Therapy
An appropriate physical
therapy program is essential to the management of any type of arthritis. A physical
therapist will explain the importance of certain activities and recommend exercises
suited to your child's specific condition. The therapist may recommend range-of-motion
exercises to restore flexibility in stiff, sore joints and other exercises to help
build strength and endurance.
Regular Exercise
When pain strikes, it's natural for your child to want to sit still. But it's important
to maintain a regular exercise program. Muscles must be kept strong and healthy so
they can help support and protect joints. Regular exercise also helps to maintain
range of motion.
At home and at school, your child should have regular exercise
and physical fitness programs. Safe activities include walking, swimming, and bicycling
(especially on indoor stationary bikes). Be sure that your child warms up the
muscles through stretching before exercising. Make exercise a family activity to build
fun and enthusiasm.
Ask the doctor and physical therapist about sports restrictions. Some, especially
impact sports, can be hazardous to weakened joints and bones. And make sure your child
eats a balanced diet that includes plenty of calcium
to promote bone health.
Date reviewed: April 2016
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