Hughes Syndrome:
Antiphospholipid Antibody Syndrome
The antiphospholipid antibody syndrome, also known as Hughes Syndrome, is a disorder characterized by multiple different antibodies that are associated with both arterial and venous thrombosis (clots). There are three primary class of antibodies associated with the antiphospholipid antibody syndrome: 1) anticardiolipin antibodies, 2) the lupus anticoagulant and 3) antibodies directed against specific molecules including a molecule known as beta-2-glycoprotein 1.
Historically, antiphospholipid antibodies were first noted in patients who had positive tests for syphilis without signs of infection. Subquently, a clotting disorder was associated with two patients with systemic lupus erythematosus in 1952. In 1957 a link between recurrent pregnancy loss and what is now called the lupus anticoagulant was established. Ultimately, the lupus anticoagulant was further described in 1963 and in 1972 the term lupus anticoagulant was given. In 1983, Dr. Graham Hughes described the association between antiphospholipid antibodies and arterial as well as venous thrombosis.
抗疫志愿者There are two main classifications of the antiphospholipid antibody syndrome. If the patient has an un
derlying autoimmune disorder, such as systemic lupus erythematosus, the patient is said to have condary antiphospholipid antibody syndrome. If the patient has no known underlying autoimmune disorder, it is termed primary antiphospholipid antibody syndrome.
Mechanism of the Antiphospholipid Antibody Syndrome:
The antiphospholipid antibody syndrome is an autoimmune phenomenon. The immune system’s function is to watch for and defend against foreign substances in the human body (for instance, bacteria or virus). One component of this defence system is the antibody. An antibody is a protein that can recognize and bind to a foreign substance. Once it has bound to this substance, it can attract other molecules and cells to destroy the offending molecule.
In some dia states, the immune system is not able to differentiate between foreign invading substances and normal components of the body; this is referred to as autoimmunity. There are a number of well known autoimmune disorders, including systemic lupus erythematosus, and studies on other dias have suggested autoimmune components in a number of other illness.
In the antiphospholipid antibody syndrome, the body produces antibodies that recognize various molecules in the body that, under normal circumstances, it would not. The molecules (phospholipids for example) play a role in the clotting process at the level of both cells (platelets) and proteins (the clotting factors); the molecules also have other functions outside of their role in clotting. For reference, the diagram on the previous page shows the general process by which clotting occurs and the diagram below is a depiction of the clotting cascade. The exact mechanism by which the antiphospholipid antibodies and anticardiolipin antibodies induce thrombophilic state is not known. A great deal of rearch is being done to explore the interactions the antibodies have with the components of the coagulation cascade and ultimately their role in the hypercoaguable state. At this time, there are numerous theories as to how the antibodies cau a hypercoaguable state; each of the theories has supporting evidence and evidence that calls it into question.
可爱的布娃娃Epidemiology of the Antiphospholipid Antibody Syndrome:
The prevalence in the general population is around 2-4%.
笋干的做法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.临榆
In patients with an initial prentation of primary antiphospholipid antibody syndrome, around 10% will eventually go on to be diagnod with an autoimmune disorder such as systemic lupus erythematosus or a mixed connective tissue disorder.
Risks of the Antiphospholipid Antibody Syndrome:
The role of the antiphospholipid antibody syndrome in both arterial and venous thrombotic disorders is an active area of clinical rearch. To date, studies examining the role of the antiphospholipid antibody syndrome in thrombosis are numerous. Clearly, the antiphospholipid antibody syndrome is associated with both arterial and venous thrombosis. However, a review of the literature clearly demonstrates continued controversy regarding the degree of risk the antibodies confer. Studies have not shown any clear differences between patients with the primary antiphospholipid antibody syndrome versus the condary antiphospholipid antibody syndrome.
A risk of recurrent thrombi, both arterial and venous, is associated with the antiphospholipid antibody syndrome as well. Most studies suggest that patients who have a recurrent episode will have it in a similar blood vesl type. In other words, patients who have a stroke initially will most often have a stroke if they have a recurrence. None-the-less, patients are reported that have multiple different types of thrombotic events.
The antiphospholipid antibody syndrome is also associated with miscarriages as well as other complications of pregnancy including preterm labor and preeclampsia. An association with thrombocytopenia (low platelets) has also been established. This occurs in 20-40% of patients with the antiphospholipid antibody syndrome.
Treatment of the Antiphospholipid Antibody Syndrome:
Studies of the optimal treatment for the antiphospholipid antibody syndrome are currently under way. Treatment of the initial thrombosis in patients with the antiphospholipid antibody syndrome does not generally differ from treatment of patients with the same disorder who do not have the antiphospholipid antibody syndrome. Anticoagulation with heparin and then subquently with oral anticoagulation is initiated. The duration of anticaogulation in patients without the antiphospholipid antibody syndrome is generally 3-6 months. In patients with the antiphospholipid antibody syndrome, the risk of recurrence is relatively high for both arterial and venous thrombotic events. As a result, patients are generally started on long-term (in some cas life-long) oral anticoagulation. The treatment of women who are pregnant and have the antiphospholipid antibody syndrome can result in a much higher success rate for the pregnancy. Several regimens have been studied including heparin.
The role of medications generally ud in autoimmune disorders to try and control the immune system is extremely limited. The primary role of the medications is in patients who have condary antiphospholipid antibody syndrome, and they generally have no effect on the antiphospholipid antibody syndrome, but can help control the systemic lupus erythematosus, for exa
mple. Anti-platelet drugs, such as aspirin, are also ud. At this time, a large study looking at the u of aspirin versus oral anticoagulation is underway in patients with the antiphospholipid antibody syndrome and stroke. U of low-molecular-weight heparins instead of warfarin or in combination with other medications is also sometimes ud.
In the ca of patients who are discovered to have the antiphospholipid antibodies without any known thrombotic problems, the question of preventative (prophylactic) treatment is unresolved. Currently, aspirin is the general recommendation.思想汇报学习方面
The u of long-term anticoagulation has risks associated with it (approximately a 3% chance per year of having a major hemorrhage, of which approximately 1/5 are fatal). Beginning long-term anticoagulation is influenced by the patient’s overall risk of recurrent thrombosis balanced against the risks associated with long-term anticoagulation on an individual basis.
Pregnancy and the Antiphospholipid Antibody Syndrome:
As mentioned above, the antiphospholipid antibody syndrome is associated with complications in pregnancy. The complications can include miscarriages, preterm labor, low birth-weight and preeclampsia. For women with known antiphospholipid antibody syndrome, it is recommended that
pre-pregnancy counling is obtained. This allows the patient to be monitored cloly from the beginning of the preganncy. Treatments during pregnancy are a subject of active investigation at this time. Currently, veral studies have examined the u of heparin along with low-do aspirin (dos are generally that of a baby aspirin) throughout the pregnancy and have demonstrated improved fetal outcomes. Other regimens that have been examined the u of aspirin and prednisone. Further studies of aspirin and prednisone combinations suggested that complications associated with prednisone u, in most cas, outweight the benefits and thus prednisone is not a commonly ud agent in addition to aspirin.
高山流水比喻什么牙膏成分In patients for whom the above treatments are not successful, u of intravenous immune globulin (IVIG) has been ud. At this time, the studies suggest this may be helpful in refractory cas, but is not recommended for u on a routine basis.
飞蛾为什么扑火Further Information:
For further information, plea visit the University of Illinois – Urbana/Champaign and Carle Cancer Center webpages on coagulation disorders at:
This information is provided as a resource to patients and health care providers. The information contained above reprents common diagnostic and treatment modalities, but
