Antiphospholipid Antibody Syndrome

Antiphospholipid syndrome (APS or APLS) or antiphospholipid antibody syndrome is a rare disorder of coagulation, which causes blood clots (thrombosis) in both arteries and veins, as well as pregnancy-related complications such as miscarriage, preterm delivery, or severe preeclampsia. The syndrome occurs due to the autoimmune production of antibodies against phospholipid (aPL), a cell membrane substance. In particular, the disease is characterised by antibodies against cardiolipin (anti-cardiolipin antibodies) and β2 glycoprotein.

The term “primary antiphospholipid syndrome” is used when APS occurs in the absence of any other related disease. APS is commonly seen in conjunction with other autoimmune diseases; the term “secondary antiphospholipid syndrome” is used when APS coexists with other diseases such as systemic lupus erythematosus (SLE)*. In rare cases, APS leads to rapid organ failure due to generalized thrombosis and a high risk of death; this is termed “catastrophic antiphospholipid syndrome” (CAPS).

Antiphospholipid syndrome is sometimes referred to as Hughes syndrome after the rheumatologist Dr. Graham R.V. Hughes (St. Thomas’ Hospital, London, UK) who worked at the Louise Coote Lupus Unit at St Thomas’ Hospital in London.

June is APS Awareness Month

Visit the APS Foundation of America:
http://www.apsfa.org/

Visit the Hughes Syndrome Foundation:
http://www.hughes-syndrome.org/

Visit the Antiphospholipid Antibody Support Group
http://www.egroups.com/group/aplsuk

Epidemiology of the Antiphospholipid Antibody Syndrome:
from http://www.med.illinois.edu/hematology/PtAPS.htm

  • The prevalence in the general population is around 2-4%.
  • Of patients with the antiphospholipid antibody syndrome, over half (50%) of them have the primary antiphospholipid antibody syndrome.
  • In persons with systemic lupus erythematosus, around 30% will develop the antiphospholipid antibody syndrome.
  • In general, anticardiolipin antibodies are more common that the lupus anticoagulant; anticardiolipin antibodies occurs approximately 5 times more often than the lupus anticoagulant in patients with the antiphospholipid antibody syndrome.
  • In patients with an initial presentation of primary antiphospholipid antibody syndrome, around 10% will eventually go on to be diagnosed with an autoimmune disorder such as systemic lupus erythematosus or a mixed connective tissue disorder.

Treatment

There’s no cure for antiphospholipid syndrome, but medications can be effective in reducing your risk of blood clots. Often, this disease is treated by giving aspirin to inhibit platelet activation, and/or Warfarin as an anticoagulant. The goal of the prophylactic treatment is to maintain the patient’s INR between 3.0 – 4.0. It is not usually done in patients who have not had any thrombotic symptoms. During pregnancy, low molecular weight heparin and low-dose aspirin are used instead of Warfarin because of Warfarin’s teratogenicity. Women with recurrent miscarriage are often advised to take aspirin and to start low molecular weight heparin treatment after missing a menstrual cycle. In refractory cases plasmapheresis may be used.

For additional Information click these links:
American Autoimmune Related Diseases Association, Inc.
AutoImmunity Community
Autoimmune Information Network, Inc
Lupus Foundation of America, Inc.

lupus1

*Maybe it is…

Systemic lupus erythematosus is a chronic autoimmune connective tissue disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body’s cells and tissue, resulting in inflammation and tissue damage. The rate of SLE varies considerable between countries, ethnicity, by gender, and has changed over time. In the United States the prevalence of SLE is estimated to be about 53 per 100,000, translating to about 1 million people in the US being affected.

The course of the disease is unpredictable, with periods of illness (called flares) alternating with remissions. The disease occurs nine times more often in women than in men, especially between the ages of 15 and 50, and is more common in those of non-European descent.

SLE is treatable through addressing its symptoms, mainly with corticosteroids and immunosuppressants; there is currently no cure. SLE can be fatal, although with recent medical advances, fatalities are becoming increasingly rare. Survival for people with SLE in the United States, Canada, and Europe is approximately 95% at five years, 90% at 10 years, and 78% at 20 years.

The S. L. E. Lupus Foundation

Lupus as a Cause of Stroke; Relationship with APS

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~ by marbleroad on June 10, 2009.

2 Responses to “Antiphospholipid Antibody Syndrome”

  1. I have lupus and have three blood clotting disorders along with it! Interesting reading here!

  2. It is so good, not only to have a name to put against my illness, but to have an explanation of past miscarriages, and now an understand of what is happening – just very sad I found out after the damage had been done.

    A very informative blog, thank you 🙂

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