Juvenile Rheumatoid Arthritis- Clinical Features And Complications.

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JUVENILE RHEUMATOID ARTHRITIS:

The American College of Rheumatology has defined it as arthritis

of one or more joints with onset below the age of 16 years and persisting

for atleast 6 weeks with explicit exclusion of other specific diseases such

as juvenile ankylosing spondylitis, juvenile psoriatic arthritis, infectious

and post infectious arthritis and other rheumatic diseases. Prevalence is

in the range of 0.4 – 1.3 per 1000 children.

Juvenile Rheumatoid Arthritis is an autoimmune disease with

major histocompatibility complex (MHC) associated with genetic

predisposition. The classical IgM, Rheumatoid Factor is usually negative

but is believed that some children have hidden IgA Rheumatoid Factor.

CLINICAL FEATURES:

Three major types of onset are described according to the

presentation during first 6 months of disease.

1. Pauciarticular onset JRA:

This accounts for about 60% of patients. Four or less joints usually

large are involved in an asymmetrical fashion. Joint swelling rather than

joint pain is the usual complaint.

2. Polyarticular onset JRA:

Occurs in 30% of patients and is common in girls. Five or more

joints (both large and small) are affected within the first 6 months ofonset. Increased joint pain in proportion to the degree of joint swelling is

the usual complaint. Fever and malaise can be significant.

3. Systemic onset JRA:

About 10% of patients with JRA may have an acute onset of the

disease with prominent systemic features that may precede the joint

manifestation. Illness usually begins as an intermittent fever with a

characteristic peak, twice daily. This is accompanied by a characteristic

maculopapular rash with central clearing. Hepatosplenomegaly and

lymphadenopathy are common. Pericarditis and interstitial lung disease

may be present. Antinuclear antibodies are positive but rheumatoid

factor is negative.
COMPLICATIONS:

RA is not fatal, but complications of the disease may shorten

survival by a few years in some individuals. In general, RA is progressive

and cannot be cured. In some, the disease gradually becomes less

aggressive and symptoms may even improve. However, if bone and

ligament destruction and any deformities have occurred, the effects are

permanent. According to one survey, 70% of patients with RA believe

that the disease prevents them from living a fully productive life.

Lymphoma and other cancers: Alterations in the immune system

associated with RA may play a role in the higher risk for lymphoma

observed in patients with RA. Aggressive treatments for RA that suppress

the immune system may help preventing this cancer, but more research is

needed to evaluate this possibility. Other cancers that may occur with

increased frequency in patients with RA include prostate and lung

cancers.

Macrophage activation syndrome: This is a life-threatening

complication of RA and requires immediate treatment. Patients should be

aware of symptoms, which include persistent fever, weakness,

drowsiness, and lethargy.

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