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Juvenile Rheumatoid Arthritis (JRA) is a chronic inflammatory disease that always involves the joints, but may produce extensive connective tissue and visceral lesions.
Incidence; Causes & Development
JRA is arbitrarily defined as beginning before the age of 16 years. Systemic onset JRA does not have a peak age for occurrence in childhood, and appears to effect males and females with equal frequency.
Despite extensive research the cause of this condition remains unclear.
Signs & Symptoms
Approximately 20% have acute systemic JRA which is characterized by febrile onset, variable joint manifestations, rash, generalized lymphadenopathy, splenomegaly, liver disease and, rarely, by GI tract disease. Other children with JRA primarily have arthritis that is present at the onset of illness. In 30% only a single joint, usually the knee, is involved; in 50% multiple joints are involved. The disease is termed polyarticular if more than 4 joints are involved and pauciarticular if 4 or fewer joints are affected. The acute systemic onset of JRA is often referred to as Still's disease because of Still's classic description of the disease in 1897.
The systemic symptoms of JRA may precede the onset of overt arthritis for a variable periods of time ranging from months to years. The most characteristic features of systemic JRA are a high-spiking fever and the rheumatoid rash. The temperature peaks once or twice daily, often in the late afternoon or evening, to a level of 39°C or higher with a rapid return to baseline which might be a sub-normal temperature. Chills are frequent at the time of the fever though rigors rarely occur. These children often appear quite ill while febrile but surprisingly well during the rest of the day. These fevers may respond poorly to anti-pyretics (including aspirin). In some instances, the fever responds only to steroid therapy.
The fever in cases of systemic JRA is almost always accompanied by the classic rheumatoid rash. This consists of 2-5mm erythematous morbilliform macules most commonly seen on the trunk and proximal extremities though it may occur on the face, palms, or soles. The rash is non-pruritic but most characteristic is its transient and migratory nature. A single lesion rarely persists for more than an hour. The rash may be seen in a small number of patients with polyarthritis at onset but never with oligoarthritis without other systemic symptoms. The rash can be elicited by rubbing or scratching the skin - Koebner's phenomenon - or may be elicited during a hot bath or with psychological stress.
Children with the systemic onset of JRA usually have lymphadenopathy and hepatosplenomegaly accompanying their active disease. Other organ systems may become involved (i.e. pericarditis or, much less commonly, hepatitis, pulmonary interstitial fibrosis or central nervous system involvement).
Diagnosis & Tests
The presence of arthritis must be confirmed to make a definite diagnosis of JRA and thus usually occurs within the first year of suspected diagnosis.
Laboratory tests are not helpful in confirming the diagnosis of Still's Disease. Both the ANA and Rheumatoid Factor are usually negative and there is no association with HLA-B27. Antinuclear antibody may be positive, if so, it is often in a speckled pattern. A positive ANA is found in young females with pauciarticular arthritis who are at risk for developing iridocyclitis.
The differential diagnosis includes acute and chronic infection, malignancy, Kawasaki's disease, inflammatory bowel disease, rheumatic fever, and SLE.
Prognosis; Complications
About half the children with systemic onset JRA recover nearly completely in time while the other half show progressive involvement of more joints with moderate to severe disability.
Chronic uveitis is a perplexing complication of JRA which occurs most commonly in young girls with oligoarthritis and ANA seropositivity. Around 1/4 of patients with oligoarthritis develop uveitis. In children with chronic uveitis approximately 60% have complete recovery of normal sight, 25% have impaired vision or unilateral blindness, and approximately 10% are blind. Children with systemic JRA rarely develop chronic uveitis (<1%).
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Recommendations and treatments for Juvenile Rheumatoid Arthritis: | | | | | Diet |  Nightshade Family Food Avoidance | Elimination of nightshade family foods may help a small percentage of those with juvenile arthritis. |
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Drug |
 Aspirin| | Aspirin is the preferred drug for therapy given as 80-100mg/kg/day. Higher doses are usually reserved for children with more severe disease. Serum salicylate levels should be maintained between 20 and 30mg/dl. Liver enzymes should be monitored prior to and during aspirin therapy. Some elevation of liver enzymes almost always occurs. Treatment, in the absence of overt side-effects, should be continued for 6 months beyond any indication of active disease and therapy should be tapered slowly. Prednisone in a dose of 1mg/kg/day may be used for brief periods. Physical therapy of the involved joints is essential in preventing long-term disability. |
NSAIDs| | Indomethacin is frequently more effective than aspirin in treating individuals with pauciarticular arthritis of the lower extremities and ankylosing spondylitis. The recommended dose is 1.5-3mg/kg/day and should not exceed 250mg/day. |
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Lab Tests/Rule-Outs |
Bacteria / Pathogen Testing by PCR |
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KEY | | May do some good | | | Highly recommended |
GLOSSARY
Acute
An illness or symptom of sudden onset, which generally has a short duration.
Antibody (Antibodies)
A type of serum protein (globulin) synthesized by white blood cells of the lymphoid type in response to an antigenic (foreign substance) stimulus. Antibodies are complex substances formed to neutralize or destroy these antigens in the blood. Antibody activity normally fights infection but can be damaging in allergies and a group of diseases that are called autoimmune diseases.
Arthritis (Arthritic)
Inflammation of a joint, usually accompanied by pain, swelling, and stiffness, and resulting from infection, trauma, degenerative changes, metabolic disturbances, or other causes. It occurs in various forms, such as bacterial arthritis, osteoarthritis, or rheumatoid arthritis. Osteoarthritis, the most common form, is characterized by a gradual loss of cartilage and often an overgrowth of bone at the joints.
Autoimmune Disease (Autoimmune, Autoimmunity)
One of a large group of diseases in which the immune system turns against the body's own cells, tissues and organs, leading to chronic and often deadly conditions. Examples include multiple sclerosis, rheumatoid arthritis, systemic lupus, Bright's disease and diabetes.
Central Nervous System (CNS)
A collective term for the brain, spinal cord, their nerves, and the sensory end organs. More broadly, this can even include the
neurotransmitting hormones instigated by the CNS that control the chemical nervous system, the endocrine glands.
Chronic (Chronicity)
Usually referring to chronic illness: Illness extending over a long period of time.
Deciliter (dL)
0.1 or one tenth of a liter.
Enzymes (Enzyme)
Specific protein catalysts produced by the cells that are crucial in chemical reactions and in building up or synthesizing most compounds in the body. Each enzyme performs a specific function without itself being consumed. For example, the digestive enzyme amylase acts on carbohydrates in foods to break them down.
Epstein Barr Virus (EBV, Epstein-Barr Virus)
A virus that causes infectious mononucleosis and that is possibly capable of causing other diseases in immunocompromised hosts.
Febrile
Having to do with a fever.
Gastrointestinal (GI, GI Tract)
Pertaining to the stomach, small and large intestines, colon, rectum, liver, pancreas, and gallbladder.
Hepatitis
Inflammation of the liver usually resulting in jaundice (yellowing of the skin), loss of appetite, stomach discomfort, abnormal liver function, clay-colored stools, and dark urine. May be caused by a bacterial or viral infection, parasitic infestation, alcohol, drugs, toxins or transfusion of incompatible blood. Can be life-threatening. Severe hepatitis may lead to cirrhosis and chronic liver dysfunction.
Kilogram (kg, kgs, Kilogramme, Kilogrammes, Kilograms)
1000 grams, 2.2lbs.
Lesion (Lesions)
Any damage to tissue structure or function; an abnormal change in body tissue caused by disease or injury. A scar is a lesion, as is cancer, a stomach ulcer or a pimple.
Liver (Hepatic)
The largest and one of the most complex organs of the body, the liver is responsible for much of the metabolism of fats, proteins and carbohydrates. It is the site of much of the body's detoxification. It is connected very closely with digestion and the regulation of blood sugar, among many other functions. Found behind the ribs on the right side of the abdomen, it has many important functions such as removing harmful material from the blood, making enzymes and bile that help digest food, and converting food into substances needed for life and growth. Hepatic: Pertaining to the liver.
Lung (Lungs, Pulmonary)
Organ of the body, located in the chest cavity which is designed to bring oxygen from the air into the blood stream, while also expelling carbon dioxide and other waste gases out of the body. Pulmonary: Related to the lungs.
Lymphadenopathy
A lymph gland enlargement in response to any foreign substance or disease.
Milligram (mg, Milligrams)
0.001 or a thousandth of a gram.
Millimeter (Millimeters, mm)
A metric unit of length equaling one thousandth of a meter, or one tenth of a centimeter. There are 25.4 millimeters in one inch.
Proximal
Nearer to a point of reference such as an origin, a point of attachment, or the midline of the body.
Rheumatism (Rheumatoid)
General term applied to conditions of pain, or inability to articulate, various elements of the musculoskeletal system.
Rheumatoid Arthritis
A long-term, destructive connective tissue disease that results from the body rejecting its own tissue cells (autoimmune reaction).
Serum
The cell-free fluid of the bloodstream. It appears in a test tube after the blood clots and is often used in expressions relating to the levels of certain compounds in the blood stream.
Splenitis (Splenomegaly)
Inflammation of the spleen, caused by a variety of conditions ranging from exposure to hemolytic chemicals, systemic infections lodged in the spleen, even cancer. Splenomegaly: For practical purposes a term interchangeable with splenitis, since neither will have the usual symptoms associated with inflammation. Splenomegaly is often associated with viral hepatitis, mononucleosis, typhoid fever and abnormally high levels of red blood cells or platelets.
Spondylitis
Inflammation of one or more vertebrae.
Steroid (Steroids)
Any of a large number of hormonal substances with a similar basic chemical structure containing a 17-carbon 14-ring system and including the sterols and various hormones and glycosides.
Last updated: Apr 13, 2008
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