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Antiphospholipid Antibody Syndrome (APAS)

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What is Antiphospholipid Antibody Syndrome (APAS)

Antiphospholipid antibody syndrome (APAS) is a group of clinical manifestations associated with the presence of high levels of antiphospholipid antibodies (APA) in the blood of the affected individual. It is associated with recurrent thromboses (formation of blood clots) in the venous or arterial circulation.

There are a number of classifcations of APAS:

  1. Primary: No associated disorder;
  2. Secondary: Those clearly exhibiting features of an autoimmune disease such as systemic lupus erythematosus (SLE);
  3. Catastrophic: Formation of many thrombi in multiple locations in a short space of time.

Statistics on Antiphospholipid Antibody Syndrome (APAS)

Unknown. APAs may be found in the blood of up to 50% of people affected by systemic lupus erythematosus. However these antibodies have also been found in 5-15 of apparently normal individuals.

There does not appear to be any racial predisposition for primary APAS, however it appears to be more common in young-middle aged adults.

Risk Factors for Antiphospholipid Antibody Syndrome (APAS)

As APA have been found in apparently normal individuals it seems that they are necessary, but not sufficient in themselves to cause the clinical manifestations of antiphospholipid syndrome. It is suggested that triggering factors are involved, for instance various infections or medications. A genetic association also seems likely.

Symptoms of Antiphospholipid Antibody Syndrome (APAS)

Symptoms may include:

  • Livedo reticularis (bluish mottling of the skin, usually on the legs);
  • Leg and finger ulcers;
  • Thrombophlebitis (development of blood clot and inflammation in a vein);
  • Painful bruising (purpura);
  • Leg swelling (DVT);
  • Pulmonary embolism;
  • Peripheral oedema (swelling of peripheral body parts, due to renal impairment);
  • Gangrene affecting fingers and toes;
  • Myocardial infarction (‘coronary’);
  • Atypical heart sounds (murmurs).

How is Antiphospholipid Antibody Syndrome (APAS) Diagnosed?

Normally blood tests are performed looking for abnormalities in the blood. CT scans and MRI are useful in identifying past strokes or heart attacks. Doppler studies are used to identify deep vein thrombosis (DVT).

Prognosis of Antiphospholipid Antibody Syndrome (APAS)

People with APAS are at increased risk from an early age of death or disability caused by stroke, heart attack, pulmonary embolism and renal failure. However, they are usually able to lead normal and rewarding lives with appropriate treatment. The presence of an underlying autoimmune disease such as SLE also affects outcome.

How is Antiphospholipid Antibody Syndrome (APAS) Treated?

Occasionally patients are very ill and may require hospitalisation. Usually they can be treated as outpatients. A variety of specialists may be involved in the care of one patient.

It is desirable to limit other factors which increase the tendency of the blood to coagulate, including quitting smoking, ceasing the oral controceptive pill and controlling blood pressure.

If a patient has developed thromboses in the past, they may need to take long term anticoagulant medication such as warfarin.

Patients who are pregnant need to be treated and monitored carefully throughout pregnancy due to the risk of developing complications.

Antiphospholipid Antibody Syndrome (APAS) References

  1. Belilos E, Carsons S. Antiphospholipid syndrome [online]. Omaha, NE: eMedicine; 2004 [cited December 2004]. Available from: URL link
  2. Cervera R. Coronary and valvular syndromes and antiphospholipid antibodies. Thromb Res. 2004;114(5-6):501-7. Abstract
  3. Roubey RA. Antiphospholipid antibodies: Immunological aspects. Clin Immunol. 2004;112(2):127-8. Abstract
  4. Pasquali JL, Nehme H, Korganow AS, Martin T. Antiphospholipid antibodies: Recent progresses on their origin and pathogenicity. Joint Bone Spine. 2004;71(3):172-4. Abstract
  5. Harris EN, Pierangeli SS. Primary, secondary, catastrophic antiphospholipid syndrome: Is there a difference? Thromb Res. 2004;114(5-6):357-61. Abstract
  6. Cotran RS, Kumar V, Collins T, Robbins SL. Robbins Pathologic Basis of Disease (6th edition). Philadelphia: WB Saunders Company; 1999. Book

Drugs/Products Used in the Treatment of This Disease:

Dates

Posted On: 11 September, 2003
Modified On: 19 March, 2014

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