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HUGHES
SYNDROME
Up to 50%
of patients with SLE have antibodies to phospholipids ('sticky blood').
SLE patients with
Antiphospholipid antibodies: 40% have thrombosis.
SLE without Antiphospholipid
antibodies: 12% to 18% of patients.
SLE patients have a higher
incidence of cardiac valvular disease, hemolytic anemia, leukopenia and low C4
complement levels.
Hemorrhagic stroke occurs
more commonly in active lupus.
Thrombotic stroke, possibly
related to the Anti-phospholipid antibody, is more common in quiescent
phases-(remissions).
Prevalence Of
Antiphospholipid Antibodies:
Presence of antiphospholipid antibodies does not
necessarily indicate an increased risk for thrombosis. The presence of AP-As
is considered a risk factor for stroke, but this association may be true only in
patients with SLE or other autoimmune disorders. Many well-controlled
studies have found no association between AP-As and stroke in non-SLE
patients.
Autoimmune diseases Percentage Relationships to
Secondary Anti-Phospholipid Syndrome (APS)
- Juvenile chronic arthritis - 55%
- SLE - 15% to 50%
- Sjogren's syndrome
- 42%
- Rheumatoid arthritis - up to 33%
- Idiopathic Thrombotic Purpura -
30%
- Posoriatic arthritis - 28%
- Scleroderma
- 25%
- Mixed
Connective Tissue Disease - 22%
- Giant cell arteritis - 20%
- Polymyalgia rheumatica -
20%
- Behcet's disease - 0% to 50%
Treatment to prevent
Thrombosis
Treatment
of patients with APL Antibody is controversial. The problem seems to be mainly an
abnormal hyper-coagulable state that predisposes to thrombosis of arteries
rather than true vasculitis. Asymptomatic patient with Antiphospholipid Antibodies:
Reduce risk factors for vascular disease. Patients with high
titers:
Avoid oral contraceptive. Life style change: Maintain ideal weight, cholesterol
level and physical activity; control blood pressure and avoid smoking.
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