Antiphospholipid syndrome ... (Antiphospholipid syndrome)
Antiphospholipid syndrome, also known as Hughes syndrome, is a process characterized by a greater tendency to thrombophilia, which can block blood vessels. In antiphospholipid syndrome, thrombosis can develop in almost any blood vessel in the body, but most often occur in the vessels of the lower extremities. If a blood clot forms in blood vessels in the brain, blood flow is impaired and can cause a stroke. Antiphospholipid syndrome is an autoimmune process that usually occurs in early to mid-adulthood, but can start at any age.
Women with antiphospholipid syndrome have an increased risk of complications during pregnancy. These complications may include preeclampsia, placental insufficiency, premature labor, or miscarriage. In addition, women with antiphospholipid syndrome have an increased risk of thrombosis during pregnancy than at other times during their lives. At birth, newborns of mothers with antiphospholipid syndrome may be small and lightweight. Thrombosis or pregnancy complication is usually the first sign of antiphospholipid syndrome. Other signs and symptoms of antiphospholipid syndrome affecting blood cells and vessels include thrombocytopenia, anemia due to hemolysis, and purplish skin discoloration (livedo reticularis) due to abnormalities in the small blood vessels of the skin. In addition, affected individuals may have skin ulcers, headaches, heart disease, or intellectual disability. Many people will also have other autoimmune conditions such as systemic lupus erythematosus.
Rarely, affected people develop thrombosis in multiple blood vessels throughout your body, which blocks blood flow in the affected organs, impairing their function and, ultimately causing organ failure. These individuals are said to have catastrophic antiphospholipid syndrome (CAPS). CAPS usually affects the kidneys, lungs, brain, heart and liver, and is fatal in more than half of affected individuals. Less than 1% of people with antiphospholipid syndrome develop CAPS.
Although the exact prevalence of antiphospholipid syndrome is unknown, it is thought to be fairly common, and may be responsible for up to 1% of all thrombosis. It is estimated that 20% of people under 50 who suffer a stroke have antiphospholipid syndrome. 10 to 15% of people with SLE have antiphospholipid syndrome. Similarly, 10 to 15% of women with recurrent miscarriages are likely to have the syndrome. Approximately 70% of people diagnosed with antiphospholipid syndrome are women.
The genetic cause of antiphospholipid syndrome is unknown. This process is associated with the presence of three abnormal antibodies in the blood: anticoagulant lupus, cardiolipin and anti ?2-glycoprotein I. The antibodies typically bind to specific foreign particles and germs, marking them for destruction, but antibodies in antiphospholipid syndrome attack normal proteins. When these antibodies bind to proteins, the proteins change shape and bind to other molecules and receptors on the cell surface. Binding to cells, particularly immune cells, activates the blood coagulation pathway and other immune responses.
Production lupus anticoagulant, cardiolipin and anti ?2-glycoprotein I may coincide with exposure to pathogens such as viruses and bacteria, which have similar antigens to normal human proteins. Exposure to these pathogens can cause the body to produce antibodies to fight the infection, but due to cross the body 's own proteins reracciones, antibodies also attack human proteins. Similar triggers can occur during pregnancy when the physiology of women, especially their immune system adapts to accommodate the developing fetus. These changes during pregnancy may explain the high rate of women affected.
Determinasdos polymorphisms in certain genes have been found in people with antiphospholipid syndrome and may predispose individuals to produce specific antibodies which contribute to thrombus formation. However, the contribution of these genetic changes in the development of the disease is unclear. People who test positive for the three antibodies, but have not had a thrombosis or recurrent miscarriages are said to be carriers antiphospholipid. These individuals are at increased risk of developing thrombosis than the general population.
Most cases of antiphospholipid syndrome are sporadic, meaning that occur in people with no history of disease in your family. Rarely, the disease has been described in families; however, no clear pattern of inheritance. Multiple genetic and environmental factors likely play a role in determining the risk of developing antiphospholipid syndrome.