Arthritis Information

What Causes Juvenile Arthritis

Most forms of juvenile arthritis are autoimmune disorders, which means that the body's immune system - which normally helps to fight off bacteria or viruses - mistakenly attacks some of its own healthy cells and tissues. The result is inflammation, marked by redness, heat, pain, and swelling. Inflammation can cause joint damage. Doctors do not know why the immune system attacks healthy tissues in children who develop JA. Scientists suspect that it is a two-step process. First, something in a child's genetic makeup gives him or her a tendency to develop JA, then an environmental factor, such as a virus, triggers the development of the disease.

Not all cases of JA are autoimmune, however. Recent research has demonstrated that some people, such as many with systemic arthritis, have what is more accurately called an autoinflammatory condition. Although the two terms sound somewhat similar, the disease processes behind autoimmune and autoinflammatory disorders are different.

When the immune system is working properly, foreign invaders such as bacteria and viruses provoke the body to produce proteins called antibodies. Antibodies attach to these invaders so that they can be recognized and destroyed. In an autoimmune reaction, the antibodies attach to the body's own healthy tissues by mistake, signaling the body to attack them. Because they target the self, these proteins are called autoantibodies.

Like autoimmune disorders, autoinflammatory conditions also cause inflammation. And like autoimmune disorders, they also involve an overactive immune system. However, autoinflammation is not caused by autoantibodies. Instead, autoinflammation involves a more primitive part of the immune system that in healthy people causes white blood cells to destroy harmful substances. When this system goes awry, it causes inflammation for unknown reasons. In addition to inflammation, autoinflammatory diseases often cause fever and rashes.

What Are Its Symptoms and Signs?

The most common symptom of all types of juvenile arthritis is persistent joint swelling, pain, and stiffness that is typically worse in the morning or after a nap. The pain may limit movement of the affected joint, although many children, especially younger ones, will not complain of pain. JA commonly affects the knees and the joints in the hands and feet. One of the earliest signs of JA may be limping in the morning because of an affected knee. Besides joint symptoms, children with systemic JA have a high fever and a skin rash. The rash and fever may appear and disappear very quickly. Systemic arthritis also may cause the lymph nodes located in the neck and other parts of the body to swell. In some cases (fewer than half), internal organs including the heart and (very rarely) the lungs, may be involved.

Eye inflammation is a potentially severe complication that commonly occurs in children with oligoarthritis but can also be seen in other types of JA. All children with JA need to have regular eye exams, including a special exam called a slit lamp exam. Eye diseases such as iritis or uveitis can be present at the beginning of arthritis but often develop some time after a child first develops JA. Very commonly, JA-associated eye inflammation does not cause any symptoms and is found only by performing eye exams.

Typically, there are periods when the symptoms of JA are better or disappear (remissions) and times when symptoms "flare,"or get worse. JA is different in each child, some may have just one or two flares and never have symptoms again, while others experience many flares or even have symptoms that never go away.

Some children with JA have growth problems. Depending on the severity of the disease and the joints involved, bone growth at the affected joints may be too fast or too slow, causing one leg or arm to be longer than the other. Overall growth also may be slowed. Doctors are exploring the use of growth hormone to treat this problem. JA may also cause joints to grow unevenly.

How Is It Diagnosed?

Doctors usually suspect JA, along with several other possible conditions, when they see children with persistent joint pain or swelling, unexplained skin rashes, and fever associated with swelling of lymph nodes or inflammation of internal organs. A diagnosis of JA also is considered in children with an unexplained limp or excessive clumsiness.

No single test can be used to diagnose JA. A doctor diagnoses JA by carefully examining the patient and considering his or her medical history and the results of tests that help confirm JA or rule out other conditions. Specific findings or problems that relate to the joints are the main factors that go into making a JA diagnosis.

Symptoms - When diagnosing JA, a doctor must consider not only the symptoms a child has but also the length of time these symptoms have been present. Joint swelling or other objective changes in the joint with arthritis must be present continuously for at least 6 weeks for the doctor to establish a diagnosis of JA. Because this factor is so important, it may be useful to keep a record of the symptoms and changes in the joints, noting when they first appeared and when they are worse or better.

Family history - It is very rare for more than one member of a family to have JA. But children with a family member who has JA are at a small increased risk of developing it. Research shows that JA is also more likely in families with a history of any autoimmune disease. One study showed that families of children with JA are three times more likely to have a member with an autoimmune disease such as rheumatoid arthritis, multiple sclerosis, or thyroid inflammation (Hashimoto's thyroiditis) than are families of children without JA. For that reason, having an autoimmune disease in the family may raise the doctor's suspicions that a child's joint symptoms are caused by JA or some other autoimmune disease.

Laboratory tests - Laboratory tests, usually blood tests, cannot alone provide the doctor with a clear diagnosis. But these tests can be used to help rule out other conditions and classify the type of JA that a patient has. Blood samples may be taken to test for anti-CCP antibodies, rheumatoid factor, and antinuclear antibodies, and to determine the erythrocyte sedimentation rate (ESR), described below.

• Anti-cyclic citrullinated peptide (anti-CCP) antibodies - Anti-CCP antibodies may be detected in healthy individuals years before onset of clinical rheumatoid arthritis. They may predict the eventual development of undifferentiated arthritis into rheumatoid arthritis.

• Rheumatoid factor (RF) - Rheumatoid factor, an autoantibody that is produced in large amounts in adults with rheumatoid arthritis, also may be detected in children with JA, although it is rare. The RF test helps the doctor differentiate among the different types of JA.

• Antinuclear antibody (ANA) - An autoantibody directed against substances in the cells' nuclei, ANA is found in some JA patients. However, the presence of ANA in children generally points to some type of connective tissue disease, helping the doctor to narrow down the diagnosis. A positive test in a child with oligoarthritis markedly increases his or her risk of developing eye disease.

• Erythrocyte sedimentation rate (ESR or sed rate) - This blood test, which measures how fast red blood cells fall to the bottom of a test tube, can tell the doctor if inflammation is present. Inflammation is a hallmark of JA and a number of other conditions.

X rays - X rays are needed if the doctor suspects injury to the bone or unusual bone development. Early in the disease, some x rays can show changes in soft tissue. In general, x rays are more useful later in the disease, when bones may be affected.

Other tests - Because there are many causes of joint pain and swelling, the doctor must rule out other conditions before diagnosing JA. These include physical injury, bacterial or viral infection, Lyme disease, inflammatory bowel disease, lupus, dermatomyositis, and some forms of cancer. The doctor may use additional laboratory tests to help rule out these and other possible conditions.
Who Treats It?

Treating juvenile arthritis often requires a team approach, encompassing the child and his or her family and a number of different health professionals. Ideally, the child's care should be managed by a pediatric rheumatologist: a doctor who has been specially trained to treat the rheumatic diseases in children. However, many pediatricians and "adult"rheumatologists also treat children with JA. Because there are relatively few pediatric rheumatologists and they are mainly concentrated at major medical centers in metropolitan areas, children who live in smaller towns and rural areas may benefit from having a doctor in their town coordinate care through a pediatric rheumatologist. Many large centers now conduct outreach clinics, in which doctors and a supporting team travel from large cities to smaller towns for 1 or 2 days to treat local patients.

Other members of your child's health care team may include:

• Physical therapist. This health professional can work with your child to develop a plan of exercises that will improve joint function and strengthen muscles without causing further harm to affected joints.

• Occupational therapist. This health professional can teach ways to protect joints, minimize pain, conserve energy, and exercise. Occupational therapists specialize in the upper extremities (hands, wrists, elbows, arms, shoulders, and neck).

• Counselor or psychologist. Being a child or adolescent with a chronic disease isn't easy, for the child or his or her family. Some children may benefit from sorting out their feelings with a psychologist or counselor trained to help children in this situation. Members of the child's family may benefit from counseling as well.

• Ophthalmologist. If your child's medications or form of arthritis can affect the eyes, catching problems early can help keep them from becoming serious. All children with JA need to have regular exams by an ophthalmologist (eye doctor) to detect eye inflammation.

• Dentist and orthodontist. Dental care can be difficult if a child's hands are so affected by arthritis that thorough brushing and flossing of the teeth becomes difficult. In addition, children with involvement of the jaw may have difficulty opening the mouth for proper brushing. Therefore, regular dental exams are important. Because JA can affect the alignment of the jaw, it is important for children with this disease to be evaluated by an orthodontist.

• Orthopaedic surgeon. For some children, surgery is necessary to help minimize or repair the effects of their disease. Orthopaedic surgeons are doctors who perform surgery on the joints and bones.

• Dietitian. For children with chronic diseases, good nutrition is particularly important. A dietitian can help design a nutritious diet that will benefit the whole family.

• Pharmacist. A pharmacist is a good source of information about medications, including possible side effects and drugs that have the potential to interact with one another. If a child has trouble swallowing large pills or taking other medication, the pharmacist may have suggestions for different ways to take the medication or may be able to formulate or help you get kid-friendly versions of some medications.

• Social worker. A social worker can help a child and his or her family deal with life and lifestyle changes caused by arthritis. A social worker also can help you identify helpful resources for your child.

• Rheumatology nurse. A rheumatology nurse likely will be intimately involved in a child's care, serving as the main point of contact with the doctor's office concerning appointments, tests, medications, and instructions.

• School nurse. For a school-age child, the school nurse also may be considered a member of the treatment team, particularly if the child is required to take medications regularly during school hours.

How Is It Treated?

The main goals of treatment are to preserve a high level of physical and social functioning and maintain a good quality of life. To achieve these goals, doctors recommend treatments to reduce swelling, maintain full movement in the affected joints, relieve pain, and prevent, identify, and treat complications. Most children with JA need a combination of medication and nonmedication treatments to reach these goals.

Following are some of the most commonly used treatments.
Treatments With Medication

• Nonsteroidal anti-inflammatory drugs (NSAIDs) - Aspirin, ibuprofen, naproxen, and naproxen sodium are examples of NSAIDs. They are often the first type of medication used. All NSAIDs work similarly: by blocking substances called prostaglandins that contribute to inflammation and pain. However, each NSAID is a different chemical, and each has a slightly different effect on the body.

Some NSAIDs are available over the counter, while more than a dozen others, including a subclass called COX-2 inhibitors, are available only with a prescription.

All NSAIDs can have significant side effects, so consult a doctor before taking any of these medications. For unknown reasons, some children seem to respond better to one NSAID than another. A doctor should monitor any child taking NSAIDS regularly to control JA symptoms as effectively as possible, at the optimal dose.

• Disease-modifying anti-rheumatic drugs (DMARDs) - If NSAIDs do not relieve symptoms of JA, the doctor is likely to prescribe this type of medication. DMARDs slow the progression of JA, but because they may take weeks or months to relieve symptoms, they often are taken with an NSAID. Although many different types of DMARDs are available, doctors are most likely to use one particular DMARD, methotrexate, for children with JA.

Researchers have learned that methotrexate is safe and effective for some children with JA whose symptoms are not relieved by other medications. 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 screening tests and doctor followup. Careful monitoring for side effects is important for people taking methotrexate. When side effects are noticed early, the doctor can reduce the dose and eliminate the side effects.

• Corticosteroids - In children with very severe JA, stronger medicines may be needed to stop serious symptoms such as inflammation of the sac around the heart (pericarditis). Corticosteroids such as prednisone may be added to the treatment plan to control severe symptoms. This medication can be given either intravenously (directly into the vein) or by mouth. Corticosteroids can interfere with a child's normal growth and can cause other side effects, such as a round face, weakened bones, and increased susceptibility to infections.

Once the medication controls severe symptoms, the doctor will reduce the dose gradually and eventually stop it completely. Because it can be dangerous to stop taking corticosteroids suddenly, it is important that the patient carefully follow the doctor's instructions about how to take or reduce the dose. For inflammation in one or just a few joints, injecting a corticosteroid compound into the affected joint or joints can often bring quick relief without the systemic side effects of oral or intravenous medication.

• Biologic agents - Children with JA who have received little relief from other drugs may be given one of a newer class of drug treatments called biologic response modifiers, or biologic agents. Five such agents - etanercept, infliximab, adalimumab, abatacept, and anakinra - are helpful for polyarthritis, extended oligoarthritis, and systemic arthritis. Etanercept, infliximab, and adalimumab work by blocking the actions of tumor necrosis factor (TNF), a naturally occurring protein in the body that helps cause inflammation. Anakinra works by blocking a different inflammatory protein called interleukin-1. Abatacept works by blocking the activation of certain inflammatory cells called T cells.

Treatments Without Medication

• Physical therapy - A regular, general exercise program is an important part of a child's treatment plan. It can help to maintain muscle tone and preserve and recover the range of motion of the joints. A physiatrist (rehabilitation specialist) or a physical therapist can design an appropriate exercise program for a person with JA. The specialist also may recommend using splints and other devices to help maintain normal bone and joint growth.

• Complementary and alternative therapies - Many adults seek alternative ways of treating arthritis, such as special diets, supplements, acupuncture, massage, or even magnetic jewelry or mattress pads. Research shows that increasing numbers of children are using alternative and complementary therapies as well.

Although there is little research to support many alternative treatments, some people seem to benefit from them. If a child's doctor feels the approach has value and is not harmful, it can be incorporated into the treatment plan. However, it is important not to neglect regular health care or treatment of serious symptoms.

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