What Is Juvenile Rheumatoid Arthritis?

Juvenile rheumatoid arthritis (JRA) is the most common childhood arthritis. It's also called juvenile idiopathic arthritis (JIA) and juvenile chronic arthritis (JCA).

What Is Juvenile Rheumatoid Arthritis?

Juvenile rheumatoid arthritis is joint inflammation and stiffness for more than 6 weeks in a child or teen aged 16 or younger. Inflammation causes redness, swelling, warmth, and soreness in the joints. Children with JRA often do not complain of joint pain.

The condition can affect any joint, and it may limit how well those joints work.

What Causes It?

JRA is an autoimmune disorder. This means that the immune system attacks healthy cells and tissues, causing inflammation.

Researchers don't know exactly why it happens. They suspect that it's a two-step process. First, something in a child’s genes makes them more likely to develop JRA. Then something else, such as a virus, triggers the disease.

How Is Juvenile Rheumatoid Arthritis Different from Adult RA?

The main difference is that some children with JRA outgrow the illness. Adults with RA usually have lifelong symptoms. Studies show that by adulthood, JRA symptoms disappear in more than half of all affected children.

What Are the Symptoms?

Besides joint pain, JRA may cause chronic fever and anemia. In some people, it can also affect the heart, lungs, eyes, and nervous system.

Flare-ups may last for several weeks. Later on, their symptoms may be less severe. Other symptoms of JRA may include:

  • Fevers of 103 F or higher
  • Pink rash that comes and goes
  • Eye inflammation
  • Joint pain or stiffness
  • Limping
  • Joint swelling
  • Being less active
  • Trouble with movements using the hands or fingers, like handwriting or coloring
  • Problems with bone development and growth

Are There Different Types of Juvenile Rheumatoid Arthritis?

Yes. There are three kinds of JRA. Each type is based on the number of joints involved, the symptoms, and the presence of certain antibodies in the blood.

  1. Pauciarticular JRA

Pauciarticular (paw-see-are-tick-you-lar) means that four or fewer joints are involved. This is the most common form. About half of all children with JRA have this type.

This type typically affects large joints, such as the knees. Girls under age 8 are most likely to develop it.

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Some children with pauciarticular JRA have special proteins in the blood called antinuclear antibodies (ANAs). They are more likely to get eye disease, such as iritis (inflammation of the iris, the colored part of the eye) or uveitis (inflammation of the inner eye). These children need to get regular eye exams by an ophthalmologist, a doctor who specializes in eye diseases.

Many children with pauciarticular disease outgrow JRA by adulthood. Still, eye problems can continue, and joint symptoms may come back in some people.

  1. Polyarticular JRA

About 30% of all children with JRA have this type, which affects five or more joints. The small joints, such as those in the hands and feet, are most commonly involved. But it can also affect large joints.

Polyarticular JRA often is symmetrical. This means it affects the same joints on both sides of the body.

Some children with polyarticular disease have an immune system substance  called rheumatoid factor in their blood. These children often have a more severe form of the disease.

  1. Systemic JRA

The classic signs of this type are joint swelling, fever, and a light pink rash. It may affect organs such as the heart, liver, spleen, and lymph nodes.

Also called “Still’s disease,” it affects 5%-10% of children with JRA.

A small percentage of children with systemic JRA develop arthritis in many joints. They may have severe arthritis that lasts into adulthood.

How Do Doctors Diagnose Juvenile Rheumatoid Arthritis?

JRA is often hard to diagnose. Sometimes children with JRA do not complain of pain. In fact, parents may not be aware of symptoms such as swelling.

Some symptoms are similar to other serious conditions such as infection, cancer, bone disorders, Lyme disease, and lupus.

If a doctor thinks a child may have JRA, she will ask about symptoms and the family’s medical history. She will give the child a physical exam to look for joint swelling, rashes, signs of internal organ inflammation, and eye problems. She may also take samples of the child’s blood and joint fluid, and use X-rays or other tests to check for joint damage and signs of inflammation.

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How Is It Treated?

Treatment usually includes medications along with exercise. The goals are to:

  • Curb inflammation
  • Ease pain and swelling
  • Strengthen joints and help them move better
  • Prevent joint damage and other complications

Which Medications Treat It?

Medications for juvenile rheumatoid arthritis include:

Nonsteroidal Anti-Inflammatory Drugs (NSAIDs)

NSAIDs are used to reduce pain, fever, and inflammation.

NSAIDs come as a liquid or pill. People typically take them from one to four times a day. Some common NSAIDs are aspirin, ibuprofen, naproxen, and tolmetin.

While NSAIDs ease pain and inflammation, they also have side effects, such as stomach pain, nausea, vomiting, headache, and anemia. Sometimes the doctor will give another stomach-protecting medication to take with the NSAIDs to make stomach bleeding less likely.

Disease-Modifying Antirheumatic Drugs (DMARDs)

These drugs can slow or stop the disease from getting worse. They also help with joint stiffness, pain, and swelling. Examples of DMARDs include hydroxychloroquine (Plaquenil), methotrexate, and sulfasalazine (Azulfidine).

It may take about 3 to 6 months for these medicines to work.

Because DMARDs are strong drugs, your child will need frequent tests to check for side effects such as anemia, low blood count, and kidney and liver problems. They may also have stomachaches, diarrhea, vomiting, nausea, headaches, rashes, loss of appetite, and weakness.

Biologic Drugs

The medicines are genetically engineered to work on the immune system. They include adalimumab (Humira), anakinra (Kineret), etanercept (Enbrel), infliximab (Remicade), and infliximab-dyyb (Inflectra), a biosimilar.

If you take them, you are more likely to get an infection, including serious diseases like tuberculosis. Some biologics are only approved by the FDA for use in adults. Check with your doctor about which ones are appropriate for children.

Corticosteroids (Steroids)

Doctors usually only prescribe these powerful anti-inflammatory drugs if other treatments don’t work or the disease is severe. They may be used early to control symptoms in systemic onset type JRA.

Steroids come in pills or liquids. When someone only has one affected joint, they may get a steroid shot.

Pills or liquids are usually prescribed for short periods of time and in low dosages. Serious side effects are linked to long-term usage. These include stunted growth, ulcers, mood swings, weight gain, "moon face," muscle weakness, high blood pressure, osteoporosis, cataracts, and infections.

Examples of steroids include dexamethasone, hydrocortisone, methylprednisolone, prednisolone, and prednisone.

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How Does Exercise Fit Into Treatment?

Exercise and physical activity are important for children with JRA. Movement brings less pain, builds strength, keeps joints moving well, and improves endurance.

With the help of physical and occupational therapists, children with JRA learn simple ways to do daily tasks. Therapists teach them exercises to help with flexibility and strengthening moves for their muscles.

It can help to use hot and cold treatments. For instance, you could use a warm compress to loosen up a stiff muscle and an ice pack to reduce swelling and inflammation.

Therapists can also make splints for children to correctly position their joints and reduce pain. People typically use them on their knees, wrists, and fingers and often use them while they sleep at night.

If your child has JRA, encourage her to get out there and be active. Avoid contact sports and instead go for things that are not stressful on the joints, like swimming.

Are There Complications With Juvenile Rheumatoid Arthritis?

There can be.

Eye problems are linked to some forms of JRA, so keep up with your child’s eye tests.

JRA can also cause problems with oral health if it affects the jaw and makes it hard to brush and floss teeth. Dentists can recommend special toothbrushes and flossing gear to help children take care of their mouths.

People with JRA often have temporal mandibular joint (TMJ) or jaw pain. Certain exercises can curb jaw pain and stiffness. If the lower jaw doesn’t develop correctly, it can cause an overbite. An orthodontist can usually fix this. In some cases, surgery may be needed.

You may also want to check with your child’s doctor about their weight. Some people with the condition may need more calories. Others may gain too much weight because they have problems being active, or because of medication side effects. Extra weight is bad for the joints.

What's the Outlook for Someone with JRA?

Most children with JRA recover from the disease fully. With proper treatment, permanent damage is now rare.

WebMD Medical Reference Reviewed by David Zelman, MD on September 01, 2016

Sources

SOURCES:

Arthritis Foundation: "Juvenile Rheumatoid Arthritis."

American College of Rheumatology: "Juvenile Idiopathic Arthritis."

National Institute of Arthritis and Musculoskeletal and Skin Diseases: "Rheumatoid Arthritis."

FDA. “FDA approves Inflectra, a biosimilar to Remicade.”

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