Juvenile Rheumatoid Arthritis

Juvenile Rheumatoid Arthritis is a disease of the immune system in children over six weeks through 16 years of age.  JRA is characterized by its attack on the joints of one’s own immune system leading to the destruction of healthy joints.  This in turn may lead to inflammation, swelling, soreness, and immobility of joints, connective tissue, and joint linings.  JRA affects between 50,000 and 100,000 children in the United States, and as many as 300,000 children in the US are afflicted with some form of arthritis.  While it is not an inherited disease, it occurs in girls more often than in boys. 

There are three main types of JRA:

Polyarticular arthritis:  symptoms include tenderness and swelling in more than

five joints accompanied by a low-grade fever.  Additionally, bumps on the body may result from sitting or leaning on various regions.  Both small joints (i.e. in the hands) and weight-bearing joints (i.e. knees) are affected

Pauciarticular JRA:  symptoms include swelling, stiffness, and tenderness in

fewer than four joints.  A unique symptom is inflammation of the iris.  This type of JRA affects most commonly the knee and wrist articulations.

            Systemic JRA:  symptoms include extensive swelling, stiffness, and pain in

numerous joints of the whole body.  Joint discomfort is accompanied by high fevers that characteristically rise in temperature during the day but then fall in temperature during the evening. 

Symptoms

            -Chronic joint pain

            -Morning stiffness

            -Joint immobility

            -Joint tenderness/swelling

            -“Hot” feeling in joints

            -Nodules or lumps under the skin

            -Associated fatigue

Diagnosis

There exists no single test for JRA, but by taking a detailed medical history and physical exam followed up with laboratory tests, blood tests, bone marrow examination, x-rays, and joint fluid and tissue tests, many conditions can be ruled out and JRA may be identified.

Treatments

JRA is most effectively treated by a combination of medication, exercise, and physical therapy.  Each treatment may be different depending on the severity of the disease and the preferences of the individual.  Outlined below are types of medications that are commonly used to relieve and manage JRA.

            Analgesics

Analgesics are medications that help with pain management by relieving mild to severe symptomatic pain, but do not necessarily treat inflammation, which is often the cause of the pain.  This type of medicine works rapidly (typically within 1 hour) to block neurological pain signals from reaching the brain.  In general, relief lasts between 4-6 hours before another dose is required to achieve equal comfort. 

Mild analgesics may be obtained without a prescription in the over-the-counter drug section in your local pharmacy.  Some familiar nonprescription analgesics include Tylenol (acetaminophen) and Advil (ibuprofen).  Care should be taken when self-administering over-the-counter medicines by noting existing medical allergies and following carefully listed dosages. 

Prescription strength analgesics may be obtained through a pharmacy with a prescription from your healthcare professional.  While these varieties offer a stronger dosage to increase comfort, they may also come with relatively more severe side effects in comparison to the over-the-counter analgesics.  The same care should be taken to notify your healthcare provider of drug allergies and to take the medicine as directed.

            Non-Steroidal Anti-Inflammatory Drugs

Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) are common drugs prescribed for pain management as well as anti-inflammatory purposes.  Thus, one would be treating the symptoms and the cause by taking an NSAID.  A familiar over-the-counter NSAID is Advil (ibuprofen), which can be obtained over-the-counter at your local pharmacy.  While an NSAID is a better alternative than a simple analgesic, it still does not affect the progression OF JRA in any way.

NSAIDs work by regulating the chemicals secreted by the body that lead to inflammation.  By controlling their production, NSAIDs lessen the pain that arises due to inflamed tissues.  However, although discomfort is reduced, traditional NSAIDs may cause side effects such as stomach ulcers and stomach lining tears, among others.  Currently, new varieties of NSAIDs are being developed so that we can reap their benefits without suffering the unwanted side effects.

            Steroids

Steroids are chemically-synthesized versions of hormones that the adrenal glands in our bodies naturally produce.  In JRA patients, they may temporarily reduce inflammation and symptomatic pain to a significant extent.  Steroids are strictly prescriptive drugs that can be administered through injections or given as pills.

While steroids offer both pain relief and inflammation reduction, there are several unwanted side effects that are associated with their use.  Possible potential medical side effects include stunted growth in children, elevated risk of infection and bruises, osteoporosis (loss of bone density, which increases susceptibility for fractures), cataracts, hypertension, blood sugar elevation, and weight gain.  All side effects are dependent on the individual’s response to the steroid, as well as dosage and length of treatment.

            Disease-Modifying Antirheumatic Drugs

Disease-modifying antirheumatic drugs may actually be able to slow the progression of JRA, perhaps to the point of actually halting the disease.  While this type of drug’s mechanism is still incompletely understood, research suggests that DMARDs inhibit inflammation of affected joints to retard the destruction of joints and cartilage. 

DMARDs are often prescribed in conjunction with NSAIDs to treat JRA more vigorously by attacking it at its root while also managing associated discomfort.  However, DMARDs may require longer periods of time to take effect.  Some DMARDs currently used to treat JRA include cyclosporine and hydroxycholoroquinine sulfate.  Like the other medical treatments, DMARDs have their own set of side effects, including eye and liver damage.

            Biological Response Modifiers

Recent research is offering a promising treatment to JRA by biologically modifying specific parts of the immune system that destroy joints.  BRMs accomplish this by blocking the action of tumor necrosis factor (TNF), a protein that is involved in the inflammation pathway.  By suppressing the effects of TNF, inflammation and destruction of the joints may decrease dramatically. 

ENBREL was the first biological response modifier that was approved to treat JRA.  In test trials, ENBREL-treated patients that were not responding to DMARD treatments showed significant improvement in their JRA progression. 

Authors: Amy Chao, B.A., UMDNJ – Robert Wood Johnson Medical School

             Ana Bracilovic, M.D., New York-Presbyterian Hospital, The University Hospital of Columbia and Cornell