The antiphospholipid antibody syndrome, also known as Hughes Syndrome. Antiphospholipid syndrome has been referred to as Hughes syndrome in honor of the doctor who first described it. It may also be associated with repeated spontaneous abortions for no apparent reason in young women. There may be a family history of blood clotting disorders in some cases.
There are two types of APS: primary and secondary. People with primary APS do not have any associated condition. The secondary form is associated with another immune disorder, such as lupus (systemic lupus erythematosus), or with a viral infection.
APS may occur in individuals with lupus or related autoimmune diseases or as a primary syndrome in otherwise healthy individuals.The syndrome can occur on its own (Primary APS) with another autoimmune disease such as Systemic Lupus Erythematosus (SLE). Sometimes the immune system doesnt function properly and makes antibodies against normal organs and tissues in the body. These self-reactive antibodies are called autoantibodies. The autoantibodies in APS were originally thought to recognize that recognize certain phospholipids, fatty molecules that make up part of normal cell membranes, hence the name antiphospholipid antibodies.
Patients with antiphospholipid antibody syndrome (APAS) tend to be under age 50 and female. Up to 2% of women may have antiphospholipid antibodies. Many will also have lupus or other autoimmune diseases.
Signs and symptoms of anti-phospholipid antibody syndrome include:
* Blood clots in the legs (deep vein thrombosis)
* Blood clots in the lungs (pulmonary embolism)
* Repeated miscarriages
* Low counts of red blood cells, white blood cells or platelets
Researchers are recently also finding that there are patients with slowly progressive memory problems and patients with a form of “atypical multiple sclerosis” and antiphospholipid antibodies detectable in their blood.
APS is diagnosed by the presence of a positive antiphospholipid antibody and either a history of blood clots in an artery or vein or a history of multiple miscarriages or other pregnancy problems.
At present, the recommended treatment is low-dose aspirin. Pregnant women are treated with either aspirin or another anticoagulant — heparin — since warfarin can cause birth defects.
In some cases, treatment may include medications that promote anticoagulation (impair clotting). Patients taking anti-coagulants need to be monitored closely given the increased risks for bleeding.
In patients for whom the above treatments are not successful, use of intravenous immune globulin (IVIG) has been used. At this time, the studies suggest this may be helpful in refractory cases, but is not recommended for use on a routine basis.