A chronic, inflammatory disease that may cause joint or connective tissue
damage. The onset occurs before Age 16. Causes, incidence, and risk factors: JRA
is thought to belong to the collagen classes of disease (those diseases that
involve connective tissue). It is a complicated disease. The primary
manifestation is arthritis, but the disease may involve other body systems such
as the heart and lining around the heart (pericardium), lungs and lining around
the lungs (pleura), eyes, and skin. Systemic arthritis affects 20% of those with
juvenile arthritis and includes fever, rash, and enlarged spleen (splenomegaly)
in addition to joint inflammation. JRA is generally divided into five broad
groups depending on whether a large number of joints are involved or just a few,
whether the rheumatoid factor (a blood test) is positive or negative, and
whether there is eye involvement or not. The five categories of JRA may be
roughly broken down as follows: ? many involved joints and a positive
rheumatoid factor ? many involved joints and a negative rheumatoid factor
? few involved joints and a positive antinuclear antibody ? few
involved joints and a positive HLA B27 surface antigen ? systemic JRA
(throughout the body) A specialist in rheumatology generally makes the category
determination. The onset of arthritis may be slow or extremely rapid. An early
sign of slow onset may be stiffness on arising in the morning. Swollen, painful
joints characterize the arthritis of JRA with pain on motion and sometimes to
touch. The skin over the joints is generally not red but can be. The systemic
form of JRA may first appear with high fevers, chills and a rash but without
joint pain. In the systemic form, arthritis may develop months after the
appearance of the fever. The two forms of JRA where there are only a few joints
involved often have associated eye disease. The most severe form of eye disease,
chronic iridocyclitis of JRA, may lead to lead to visual problems or blindness.
The milder form of JRA-associated eye disease is acute iridocyclitis, which
generally heals without permanent damage. The cause of juvenile rheumatoid
arthritis is unknown. Growth may be affected during periods of active disease.
Girls are affected more often than boys are. The peak onset occurs between the
ages of 2 to 5 years old and between 9 to 12 years old. Risk factors may be a
family history of the disease and recent rubella infection or vaccine.
Prevention: There is no known prevention for JRA. Symptoms: General symptoms:
? joint stiffness on arising in the morning ? limited range of
motion ? slow rate of growth ? hot, swollen, painful joints
? fever, low grade (with multiple-joint type disease) ? fever,
high spiking with chills (with systemic form of disease) ? rheumatoid
rash ? rheumatoid nodules (at sites of pressure) Symptoms of eye
involvement: ? red eyes ? eye pain ? photophobia ?
visual changes Other symptoms: ? chest pain ? shortness of breath
? abdominal pain Signs and tests: Physical examination may show an
enlarged liver (hepatomegaly), enlarged spleen (splenomegaly), or swollen lymph
nodes (lymphadenopathy). There may also be signs of: ? anemia ?
iridocyclitis ? pericarditis ? pleuritis ? myocarditis
? Tests include: ? CBC ? ESR ? ANA ? RA
factor ? HLA antigens ? immunoelectrophoresis – serum ?
synovial fluid analysis ? X-ray of a joint ? X-ray of the chest
? ECG ? slit-lamp exam of the eyes Treatment: Treatment is aimed
at preserving mobility and joint function and supporting the patient and family
through a long chronic illness. Therapeutic medications include: ?
aspirin ? nonsteroidal anti-inflammatory agents (NSAIDS) ?
corticosteroids ? topical ophthalmic corticosteroids ? mydriatics
? gold therapy ? chloroquine agents ? immune suppressing
agents (rarely used in children) Note: Talk to your health care provider before
giving aspirin or NSAIDS to children! Physical therapy and exercise programs may
be recommended. Surgical procedures may be indicated, including joint
replacement. Expectations (prognosis): JRA is seldom life threatening. Long
periods of spontaneous remission are typical. Often JRA improves or remits at
puberty. Approximately 75% of JRA patients eventually enter remission with
minimal functional loss and deformity. The stress of illness can often be helped
by joining a support group. Here, members share common experiences and
problems.. Complications: ? loss of vision or decreased vision ?
total joint destruction of the major weight-bearing joints ? chronic