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:

1. 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
2. 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. 
3. 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.
4. 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.
5. 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.
6. 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).
7. 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.