When 50-year-old Ann Kunkel’s daughter Kara was first diagnosed with juvenile rheumatoid arthritis (JRA), she was stunned. “I didn’t even know that kids could get arthritis,” says Ann, a mother of four from Oregon, Missouri. Now 19 years old, Kara and her 13-year-old brother Drew are among almost 50,000 American children and teens growing up with JRA.
A chronic autoimmune disorder, JRA causes joint inflammation, pain, swelling, and stiffness in children as young as a few months old. Nobody is exactly sure why the immune system goes haywire in these children, but scientists believe that the problem is caused by a combination of genetic and environmental factors. By most recent estimates, two-thirds of those diagnosed with JRA will continue to have symptoms when they are adults.
Generally, JRA is not life threatening, but chronic inflammation can damage joints and cause bones to grow abnormally. When symptoms flare, even simple tasks like tying sneakers or raising a hand to answer a question at school can require monumental effort. Fatigue, sometimes extreme, is also common.
An Elusive Diagnosis
The early signs of JRA are often subtle. Parents may notice their child limping or avoiding a favorite game. “Kara had a swollen joint here and there and a rash, but the symptoms were not continuous,” says Ann. “Finally, when she couldn’t function at school in the afternoon her teacher insisted that we take her to the doctor. He didn’t see anything wrong, but her blood work came back positive for rheumatic disease.”
There is no single test for JRA. Joint swelling and pain lasting at least 6 weeks, severe eye disease such as iritis (inflammation of the colored part of the eye, the iris), and a fever accompanied by a light pink rash are all red flags in children up to 16 years of age.
Many children do not grumble about the discomfort, either. “Kara never complained about pain. I remember once when her occupational therapist said that her ankles hurt,” says Ann. “Kara asked what that was like. After her therapist explained that they were sore and swollen, Kara said that she thought that everyone’s ankles felt like that all the time.”
Wary physicians typically order lab tests to rule out other conditions such as physical injury, bacterial infection, Lyme disease, inflammatory bowel disease, lupus, and some forms of cancer. X-rays help identify fractures and potential joint damage and check for changes in bone development.
Three Types of JRA
JRA strikes children in many different ways. Some experience minimal discomfort while others are confined to a wheelchair. It all depends on which of the three types of JRA a child has, and its severity.
* Pauciarticular Approximately half the children diagnosed with JRA have the pauciarticular form, which affects four or fewer joints. Typically, the large joints, such as the knees, ankles, and elbows on one side of the body are involved. About 20-30% of children with pauciarticular JRA develop severe eye disease, so regular visits to the ophthalmologist are critical.
* Polyarticular About 30% of children with JRA have polyarticular disease, which affects five or more joints. Although it usually involves the small joints of the fingers and hands, it can also afflict the large joints. This form often affects the same joint on both sides of the body.
* Systemic This is the least common but most severe form, affecting about 20% of children with JRA. In addition to joint pain, these children have high, spiking fevers (103 degrees F or higher) and a rash consisting of pale red spots on the child’s chest, thighs, and other parts of the body. Joint inflammation may start with the fever or begin weeks, even months, later. It can also cause dangerous inflammation of internal organs, including the heart, liver, spleen, and lymph nodes.
At present there is no cure for JRA, but early diagnosis and aggressive treatment can relieve pain, reduce swelling, minimize joint damage, and keep joints moving. “Our goal is quick, prolonged, consistent control of the inflammatory process,” says Daniel Lovell, MD, pediatric rheumatologist and researcher at Children’s Hospital Medical Center in Cincinnati, Ohio.
Many children with pauciarticular JRA find relief with nonsteroidal antiinflammatory drugs (NSAIDs) such as ibuprofen, injections of steroids directly into the joint, or a combination of the two. Children with polyarticular JRA may also start with NSAIDs, but disease-modifying antirheumatic drugs (DMARDs) are typically introduced early in treatment to slow the progress of the disease. Methotrexate, the most common DMARD, controls symptoms in about 60-70% of JRA patients.
Severe cases of systemic JRA may require the use of corticosteroids to halt life-threatening conditions such as pericarditis, an inflammation of the sac surrounding the heart. Doctors use corticosteroids cautiously since they can interfere with normal growth and cause side effects, including weakened bones and increased susceptibility to infection.
New Drug Offers Hope
This is an exciting time in rheumatology. Many promising treatments for arthritis are being developed and tested, and the US Food and Drug Administration (FDA) recently approved a new drug called etanercept (Enbrel) for the treatment of polyarticular JRA. “Before etanercept,” says Dr. Lovell, “we had few good options for children who did not respond to methotrexate. What we had was less effective and caused more side effects.”
In a study conducted by Dr. Lovell and his colleagues in the Pediatric Rheumatology Study Group, 70% of children aged 4-17 with severe arthritis showed profound clinical improvement after taking etanercept for 3 months. Some of the children began to improve as quickly as 2 weeks after starting the drug. With continued therapy, over 80% of the patients who improved with etanercept maintained their progress for more than a year.
Etanercept is not yet an accepted standard JRA treatment, however. Before it is widely distributed, further studies will have to be done to show that it works both well and safely.
Etanercept has also been effective in some patients with systemic JRA but a formal study has not been done.
Article By: Michele Turin Wick, Medical Writer