Systemic Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis (JIA) is the most common type of arthritis in children where the immune system mistakenly attacks the body’s tissues, causing inflammation in joints and potentially other areas of the body.

What is Juvenile Idiopathic Arthritis?

Nearly 300,000 children – from infants to teenagers – in the U.S. have some form of arthritis. Juvenile idiopathic arthritis (JIA) is the most common type of arthritis in children. The term idiopathic means “of unknown origin.” JIA was previously called juvenile rheumatoid arthritis, or JRA. JIA is not contagious.

There are six JIA subtypes. Systemic JIA is considered an autoinflammatory disease. The other types are considered autoimmune diseases. A healthy immune system fights invaders, such as viruses and bacteria. In people with autoimmune or inflammatory diseases, the immune system doesn’t work properly. It becomes overactive even when there is no infection to fight, or it mistakenly attacks healthy cells and tissues.

• Systemic JIA causes inflammation in one or more joints and is often accompanied by a high spiking fever (103°F or higher) that lasts at least 2 weeks and a skin rash. Other possible signs include inflammation of the heart or lungs or their outer linings; anemia; or enlarged lymph nodes, liver or spleen. About 10 percent of children with JIA will have the systemic form.

• Oligoarticular JIA causes arthritis in four or fewer joints, typically the large ones (knees, ankles, elbows). Children with oligoarthritis are more likely to get uveitis (chronic eye inflammation) than those with the other subtypes. Children with a positive test for antinuclear antibody (ANA) are at greatest risk of developing eye inflammation and will need to see an eye doctor frequently.

• Polyarticular JIA causes inflammation in five or more joints, often the small joints of the fingers and hands, but weight-bearing joints and the jaw can also be affected. Polyarthritis can be rheumatoid factor-positive or negative. Rheumatoid factor-positive polyarticular, JIA closely resembles adult rheumatoid arthritis and can be a more severe disease than the RF-negative variation. About 25 percent of children with JIA will have the polyarticular form.

• Juvenile psoriatic arthritis involves arthritis that usually occurs in combination with a skin disorder called psoriasis. The psoriasis may begin many years before any joint symptoms become apparent. Joint symptoms include pain and swelling in one or more joints, often the wrists, knees, ankles, fingers and toes. Symptoms of psoriasis include a scaling red rash commonly seen behind the ears; on the eyelids, elbows and knees; at the scalp line; or in the belly button.

• Enthesitis-related JIA is characterized by tenderness where the bone meets a tendon, ligament or other connective tissue. This tenderness, known as enthesitis, accompanies the joint inflammation of arthritis. It most often affects the hips, knees and feet. Enthesitis-related arthritis may also involve inflammation in parts of the body other than the joints. Often referred to as spondyloarthritis, enthesitis-related arthritis is more common in boys and usually begins between the ages of 8 and 15. Affected children will often test positive for the HLA-B27 gene.

• Undifferentiated arthritis is the term used to describe a juvenile arthritis that does not fit into any of the above types, or that involves symptoms spanning two or more subtypes.

Juvenile Idiopathic Arthritis Causes

Researchers are uncertain what causes JIA. There is no evidence that foods, toxins, allergies or lack of vitamins play a role in developing the disease. Current research indicates that there is a genetic predisposition to JIA. More than a dozen genetic markers have been identified for JIA, and hun­dreds more are being considered. However, genetic markers alone can’t determine who will get arthritis. Researchers believe that a trigger, like a virus, can start the disease process in those children with the genetic tendency.

Juvenile Idiopathic Arthritis Symptoms

These are some of the most common early signs and symptoms of arthritis in children. A child may not have all of these symptoms.

• Joints that are warm to the touch
• Swelling and tenderness at joints
• Fever
• Rash
• Favoring one limb over another or limping
• Pain (often worse following sleep or inactivity)
• Stiffness, especially upon waking in the morning
• Inability to bend or straighten joints completely
• Decreased physical activity
• Fatigue
• Sleep problems
• Swollen lymph nodes
• Reduced appetite and/or weight loss

Not all children with JIA will have the same symptoms, and some symptoms are specific to a subtype of JIA. Symptoms can change from day to day or throughout a single day.

It’s important to talk to a child’s doctor to ensure that these symptoms mean that a child has JIA instead of an injury or a different illness. Then parents and the doctor can begin the process of monitoring symptoms to establish a pattern. Arthritis affects every child differently, so it’s important to recognize the signs so that a timely and accurate diagnosis can be made to ensure the best possible outcome for a child’s health and well-being.

Juvenile Idiopathic Arthritis Causes

No known cause has been pinpointed for most forms of juvenile idiopathic arthritis. There is no evidence to suggest that toxins, foods or allergies cause children to develop the disease. Some research points toward a genetic predisposition, which means the combination of genes a child receives from his or her parents may cause the onset of arthritis when triggered by other factors.

Juvenile Idiopathic Arthritis Diagnosis

An early diagnosis and aggressive treatment are key to preventing or slowing joint damage and preserving joint function and mobility.

Here are some of the steps a pediatrician will perform when JIA is suspected.

• Medical history. The doctor will ask questions about the child’s health history to help determine the length of time and type of symptoms that have been present. This helps to rule out other possible causes like trauma or infections. The doctor will also ask about the family's medical history.
• Physical examination. The doctor will check the joints for swelling and redness. The child's range of motion will be test.
• Laboratory tests. The doctor may order blood tests that measure levels of certain proteins and other chemicals that are found in children with JIA. Tests may include erythrocyte sedimentation rate (ESR or sed rate), antinuclear antibody (ANA) test, anti-cyclic citrullinated peptide (anti-CCP), rheumatoid factor (RF) test, HLA-B27 typing (a genetic marker), complete blood count (CBC) and urinalysis, among others.
• Imaging. The doctor may order imaging tests, such as X-rays, ultrasound and MRI or CT scans, to identify joint damage.

The diagnosis of JIA is made by the presence of active arthritis in one or more joints for at least six weeks, after other conditions have been ruled out. The pediatrician and a pediatric rheumatologist may be involved in making the final diagnosis.

Juvenile Idiopathic Arthritis Treatment

There is no cure for JIA but with prompt diagnosis and early aggressive treatment, remission is possible. The goal of treatment is to relieve inflammation, control pain and improve quality of life. With early aggressive treatment, however, remission is possible.

The treatment plan may include medication, exercise, eye care, dental care and proper nutrition. Rarely, surgery may be necessary at later stages to help with pain or joint function.

Medication

The initial goals of drug therapy are to reduce inflammation and relieve pain. Long-range objectives are to prevent disease progression and destruction of joints, bones, cartilage and soft tissues such as muscles, tendons and joint capsules. More than one medication may be prescribed to treat a child’s disease. The idea is to hit the disease hard and fast to prevent further joint damage and stop the disease in its tracks as much as possible.

The American College of Rheumatology developed treatment recommendations for doctors who treat children with JIA. It helps doctors determine how to increase therapy quickly if current treatments aren’t effective enough. The recommendations also describe how doctors should monitor the safety and side effects of treatments.

Here are the most common classes of medications the doctor may prescribe:

• Nonsteroidal anti-inflammatory drugs (NSAIDs). These medicines include ibuprofen and naproxen and are a mainstay of early therapy for children with JA to ease pain and inflammation; however they do not prevent joint damage. Not all children respond to NSAIDs the same way, so a child’s doctor may try different ones for a child. Laboratory tests help doctors monitor side effects.
• Disease-modifying Antirheumatic Drugs (DMARDs). These are powerful anti-inflammatory medicines. They can prevent joint damage, such as cartilage and bone destruction, but can take a month or more to have an effect. They are often used in combination with other medica­tions, like NSAIDs, corticosteroids or biologics. Children taking DMARDs need regular lab tests to monitor possible side effects. Methotrexate is the most commonly prescribed nonbiologic DMARD in both children and adults with arthritis. It can be taken by mouth or by injection. It has been used for nearly 30 years to treat JIA. Other nonbiologic DMARDs used to treat JIA include sulfasalazine, leflunomide and hydroxychloroquine.
• Biologic response modifiers. Biologics are a subset of DMARDs. They are used to treat a number of autoimmune diseases including JIA. These medications help to correct a faulty response by the immune system that causes arthritis inflammation. Biologics suppress the immune system and can make a child more likely to develop infec­tions. Before a child receives a biologic, the doctor will order a tuberculosis (TB) test because biologics may activate old TB infections. Children taking biologics should not receive live vaccines such as MMR (measles/mumps/rubella) and varicella (chicken pox), which contain weakened yet active virus strains. Biologics that are FDA-approved for use in children include abatacept, adalimumab, canakinumab, etanercept and tocilizumab.
• Corticosteroids. These are strong anti-inflammatory medicines that work quickly compared to NSAIDs. Prednisone is one type. The doctor may prescribe small doses of corticosteroids to help quickly con­trol inflammation while waiting for DMARDs to take effect. Corticosteroids can be given by mouth or by injection. Injections into the joint are pre­ferred when only a few joints are involved or when a single joint is particularly bothersome. Because of the potential side effects, corticoste­roids are used at the lowest dose for the shortest length of time as possible. A child’s doctor will help determine if the benefits of treat­ment outweigh the risks.

Surgery

Surgery is rarely used to treat JIA early in the course of the disease. However, it can be used to correct leg length discrepancy, straighten a bent or deformed joint or replace a damaged joint.

Eye Care

Eye inflammation (uveitis) can occur in children with JIA, particularly in those with oligoarthritis. It is very important for children with JIA to have their eyes checked by an ophthalmologist at diagnosis and regularly as recommended by their doctor to reduce the chance of vision loss. Uveitis does not necessarily cause symptoms such as pain or red eye. The only way to confirm if uveitis is occurring is by eye examination. Uveitis inflammation can occur even if arthritis flares are under control.

Dental Care

JIA may affect the temporomandibular (jaw) joint, causing pain, stiffness and altered growth. This can make brushing and flossing difficult. A child’s dentist may suggest assistive devices or rinses to help teeth and gums stay healthy. More frequent, shorter dental visits may be necessary for children who are unable to keep their mouths open for long periods during dental work.

Splints and Orthotics

Splints help to keep joints in the correct position and relieve pain. They can be used to correct a defor­mity (bending in the wrong position). Splints are commonly used for knees, wrists or fingers. Orthotics, or shoe inserts, may help with dif­ferences in leg length and balance problems.  An occupational or physical therapist can make a custom splint for a child.

Juvenile Idiopathic Arthritis Self Care

Getting plenty of physical activity, eating well and learning how to cope with the challenges of the disease will be beneficial for children with arthritis.

Morning Stiffness Relief

Morning stiffness is one of the easiest ways to mea­sure how active a child’s arthritis is. The longer the stiffness lasts, the more active the disease. Tak­ing a hot bath or shower, sleeping in a sleeping bag or sweatsuit, doing range-of-motion exercises and using a hot or cold pack can help relieve stiffness. Although most children do better with warmth, there are a few who do well with cold treatments.

Physical Activity

Exercise is an essential part of a child’s plan. It helps keep bones and muscles strong and preserves range of motion. Physical activity will also help a child achieve and maintain a healthy weight, relieving added pressure on weight-bearing joints like knees, hips and ankles. Encourage activities such as swimming and bike riding, which exercise the joints and muscles without putting too much weight-bearing stress on the joints. Activities such as jumping on a trampoline or jogging often are not recommended. However, sports like basketball or soccer may not be off limits for a child if his or her arthritis is well controlled. Special exercises and protective equipment fur­ther reduce risk of injury. Family support can help to keep a child motivated to be physically active.

Therapeutic Exercise

Therapeu­tic exercise is the best way to preserve range of motion and strength and can help make it easier for children to perform activities of daily living. Range-of-motion exercises help with joint stiff­ness to prevent joints from becoming fixed in a bent position. Joints with poor movement are at increased risk of osteoarthritis, even if the inflam­mation is controlled. Strengthening exercises build muscle strength that can help support weak joints. A physical or occupational therapist can teach parents and a child and how to perform therapeutic exer­cises at home. Most exercises must be done daily.

Nutrition

A healthy diet is important for all children. While some people have reported improved symptom relief from eating or eliminating cer­tain foods, there is no specific diet that can cure arthritis. Following a diet low in processed foods and saturated fat and rich in fruits, vegetables, fish, nuts and beans can be beneficial for a child’s overall health and help ease inflammation.

Weight loss and gain can be associated with JIA. Weight loss may occur due to a loss of appetite. Chewing may be painful for children with af­fected jaws. Eating smaller, nutritious meals and snacks more often may help a child get the proper amount of calories. Some children with JIA may gain excess weight due to side effects of corticosteroids or limited physical activity. Being overweight puts extra stress on joints like the knees and hips. A regis­tered dietician can help parents to improve their child’s diet.

Emotional Issues

Having JIA has a big impact on a child’s life, both physically and emotionally. Helping a child learn to cope with the emotional aspects of arthritis will benefit the entire family. A parent’s attitude toward arthritis will set the tone for the child. Helping children to learn as much as possible about the disease and its treatment will help them can feel part of the decision making and in control of the disease. Parents should talk to their child about how he or she feels about arthritis and express anger or sadness. It’s probably best to try and avoid making JA the center of attention by expect a child with arthritis to behave as well as siblings and share in household responsibilities. Coping with the challenges of a chronic illness can be difficult. Professional counseling may help to facilitate the adjustment. A child’s doctor or a medical social worker can provide information about resources.