Antiphospholipid syndrome: why is it dangerous?
Only forty years ago, doctors did not even know about the existence of antiphospholipid syndrome. The discovery belongs to the physician Graham Hughes, who practiced in London. He described in detail its symptoms and causes of occurrence, which is why sometimes APS is also called Hughes syndrome.
With the development of antiphospholipid syndrome, antiphospholipid antibodies (APLA) appear in the blood, contributing to the increased formation of blood clots in the lumen of the vessels. They can complicate pregnancy and even cause it to terminate. Most often, APS is diagnosed in women aged 20-40 years.
- Pathogenesis of the development of antiphospholipid syndrome
- Features of APS diagnostics
- Reasons for the development of APS
- Types of API
- Danger of antiphospholipid syndrome
- Complications of APS
- Antiphospholipid syndrome during pregnancy
- How to detect an API during pregnancy
- What body systems are affected by APS, symptoms of disorders
- APS treatment
- Antiphospholipid antibodies in the blood - normal or pathological?
Pathogenesis of the development of antiphospholipid syndrome
In the blood of a person against the background of antiphospholipid syndrome, antibodies begin to circulate, which destroy phospholipids found in the cell membranes of the tissues of the body. Phospholipids are present in platelets, nerve cells, and endothelial cells.
Phospholipids can be neutral or negatively charged. In the latter case, they are called anionic. It is these two types of phospholipids that are found in the blood more often than others.
Since phospholipids can be different, different antibodies are produced to them. They are able to react with both neutral and anionic phospholipids.
Antiphospholipid syndrome is determined by immunoglobulins that appear in the blood during the development of the disease.
Among them are distinguished:
- Lupus immunoglobulins lgG, lgM. For the first time, these antibodies were detected in patients with lupus erythematosus. At the same time, it was possible to detect an increased tendency to thrombosis in them.
- Antibodies to cardiolipin antigen. This component of the test detects syphilis in a person. At the same time, antibodies of class A, G, M will circulate in his blood.
- Antibodies that are represented by a combination of cardiolipin, phosphatadylcholine and cholesterol. They are able to give a positive result when carrying out the Wasserman reaction (diagnosis of syphilis), but this result is false.
- Total immunoglobulins of classes A, G, M (beta-2-glycoprotein-1-cofactor-dependent antibodies to phospholipids). Since beta-2-glycoprotein-1 are phospholipids-anticoagulants, the appearance of antibodies directed to their destruction to the blood leads to increased formation of blood clots.
Detection of antibodies to phospholipids makes it possible to diagnose antiphospholipid syndrome, the detection of which is associated with a number of difficulties.
Features of APS diagnostics
Antiphospholipid syndrome gives a number of pathological symptoms that suggest this disorder. However, in order to make the correct diagnosis, laboratory tests will be required. And there will be a considerable number of them. This includes donating blood for general and biochemical analysis, as well as performing serological tests that make it possible to detect antibodies to phospholipids.
The use of one research method is not enough. Often, patients are prescribed an analysis for the Wasserman reaction, which is capable of giving a positive result not only with antiphospholipid syndrome, but also with other diseases. This leads to a misdiagnosis.
To minimize the likelihood of a medical diagnostic error, a comprehensive examination should be prescribed to a patient with APS symptoms, which should include:
- The detection of lupus antibodies is the first test that is done if an APS is suspected.
- Detection of antibodies to cardiolipin antigen (Wasserman reaction). With APS, the test will be positive.
- Test for beta-2-glycoprotein-1-cofactor-dependent antibodies to phospholipids. The values of these antibodies will exceed the acceptable limits of the norm.
If antibodies appeared in the blood earlier than 12 weeks before the manifestation of the first symptoms of APS, then they cannot be considered reliable. Also, on their basis, they do not confirm the diagnosis of APS if the tests became positive only 5 days after the onset of the disease. So, in order to confirm the diagnosis of "antiphospholipid syndrome", the presence of symptoms of the disorder and a positive test for antibodies are required (at least one study must give a positive reaction).
Additional diagnostic methods that a doctor may prescribe:
- Analysis of false positive Wasserman reaction.
- Conducting the Kumbas test.
- Detection of rheumatoid factor and antinuclear factor in the blood.
- Determination of cryoglobulins and titer of antibodies to DNA.
Sometimes doctors, with suspicion of APS, limit themselves to taking blood for the detection of lupus anticoagulant, but in 50% of cases this leads to the fact that the violation remains unidentified. Therefore, if there are symptoms of pathology, the most complete study should be carried out. This will allow early detection of APS and initiation of therapy. By the way, modern medical laboratories have tests that make it possible to carry out precisely complex diagnostics, since they are equipped with all the necessary reagents. By the way, some of these systems use snake venom as ancillary components.
Reasons for the development of APS
Antiphospholipid syndrome is most often manifested against the background of such pathologies as:
- Systemic lupus erythematosus.
- Systemic scleroderma, rheumatoid arthritis, Sjogren's syndrome.
- Cancer tumors in the body.
- Lymphoproliferative diseases.
- Autoimmune thrombocytopenic purpura, which can be triggered by the lupus erythematosus system, scleroderma, or rheumatoid arthritis. The presence of purpura significantly increases the risk of developing antiphospholipid syndrome.
HIV infection, mononucleosis, hepatitis C, endocarditis, malaria. APS can develop in viral, bacterial and parasitic infections.
- Diseases affecting the central nervous system.
- The period of bearing the child, childbirth.
- There may be a hereditary predisposition to the APS. At the same time, the phenotype of human blood will have specificities of DR4, DR7, DRw53.
- Taking certain medications, such as psychotropic drugs, birth control pills, and oral contraceptives.
The longer antiphospholipid antibodies are present in the blood, the faster a person will develop APS. Moreover, the reason for their appearance does not matter.
Types of API
The following types of API are distinguished:
- Primary antiphospholipid syndrome, which develops on its own, that is, its onset is not preceded by any disease.
- Secondary antiphospholipid syndrome, which develops against the background of autoimmune pathology, for example, with systemic lupus erythematosus.
- Catastrophic antiphospholipid syndrome, which is rarely diagnosed, but this form of pathology is extremely dangerous. The disease develops rapidly and leads to the formation of blood clots in all vessels of the body. Often this syndrome becomes the cause of death.
- AFLA is a negative syndrome that is difficult to detect. In this form of the disease, there are no lupus antibodies and antibodies to cardiolipin in the blood.
Sneddon's syndrome is a disease that develops in the presence of APS. In this case, a person has episodes of cerebral thrombosis. Symptoms of the disorder are cyanotic skin and high blood pressure. Sneddon's syndrome is referred to as one of the possible variants of the course of the disease.
Danger of antiphospholipid syndrome
Antibodies to phospholipids, which appear with APS, disrupt the normal functioning of the hemostatic system. This leads to the fact that blood clots begin to form in the vessels, a person develops thrombosis.
With APS, not only capillaries are affected, but also large vessels. In general, blood clots can form in any vein or artery that carries blood to various organs. Therefore, the symptoms of this disorder are very diverse.
Complications of APS
Antiphospholipid syndrome provokes the formation of blood clots in the vessels. Most often, the veins of the lower extremities suffer from thrombosis. If a thrombus breaks off, then with the blood flow it enters the vessels that feed the lung tissue. This leads to the development of a dangerous condition called pulmonary embolism. If a thrombus blocks the great vessel of the lung, then its infarction occurs, and cardiac activity stops. Often, PE ends in death of the patient, and death can occur very quickly.
When a blood clot blocks small vessels, a person has a chance to recover, but at the same time he must be urgently taken to a medical facility. Even so, the likelihood of severe health consequences is extremely high.
Blood clots with APS can form in the renal arteries. Against the background of such thrombosis, severe renal pathologies develop, for example, Budd-Chiari syndrome.
Less often, blood clots form in the capillaries of the retina, in the subclavian veins, in the central veins of the adrenal glands, which leads to the development of insufficiency of these organs. Also, against the background of thrombosis, the development of the inferior or superior vena cava syndrome is possible.
Thrombosis with blockage of arteries of different localization can lead to heart attack, gangrene, necrosis of the femoral head.
Antiphospholipid syndrome during pregnancy
The development of antiphospholipid syndrome during pregnancy can lead to such serious consequences as:
- Miscarriage in early pregnancy. The risk of spontaneous abortion is the higher, the more antibodies to the cardiolipin antigen circulate in the woman's blood.
- The development of placental insufficiency, which leads to fetal hypoxia with a delay in its development. If medical care is not provided, there is a high risk of intrauterine death of the child.
- Development of gestosis with eclampsia and preeclampsia.
- Chorea development.
- The formation of blood clots.
- Increased blood pressure.
- Development of HELLP-syndrome with hemolysis, damage to the liver parenchyma and thrombocytopenia.
- Premature placental abruption.
- Early childbirth.
Against the background of APS, attempts to conceive a child by in vitro fertilization may end in failure.
How to detect an API during pregnancy
Women who are at risk of developing APS should be monitored by a doctor.
According to the indications, they can be assigned the following diagnostic procedures:
- Regular hemostasiogram.
- Unscheduled ultrasound of the fetus with Doppler ultrasound of the uteroplacental blood flow.
- Ultrasound of the vessels of the legs, head, neck, kidneys, eyes.
- An echocardiogram to check if the heart valves are working.
This will make it possible to timely detect the development of serious complications of pregnancy, such as: disseminated intravascular coagulation, purpura, HUS.
In addition to a gynecologist, a pregnant woman diagnosed with antiphospholipid syndrome may need to consult other narrow specialists, for example, a rheumatologist, cardiologist, neurologist, etc.
Treatment is reduced to taking glucocorticosteroids and antiplatelet drugs. The dose should be adjusted by the doctor. Heparins and immunoglobulins may also be prescribed. These drugs are administered while monitoring the blood count.
If a woman already suffers from APS, but does not plan pregnancy, then she should not use hormonal drugs for contraception. Otherwise, you can aggravate the course of the disease.
What body systems are affected by APS, symptoms of disorders
Antiphospholipid syndrome is associated with the risk of developing various diseases. Moreover, any organs and systems, even the brain, can be affected. If its vessels are damaged, a transient ischemic attack or heart attack may develop.
This is accompanied by symptoms such as:
- The appearance of seizures.
- Dementia, which is constantly progressing.
- Mental disorders.
Also, APS can manifest itself with the following neurological symptoms:
- Severe migraine-type headaches.
- Uncontrolled trembling of the limbs.
- Symptoms characteristic of transverse myelitis. They arise because the spinal cord suffers from APS.
The most formidable complication of heart damage is a heart attack. It develops when blood clots form in the coronary arteries. If their small branches are involved, then the heart attack will be preceded by violations of the heart contraction. Also, APS can lead to the development of heart disease, to the formation of an intracardiac thrombus. Such indirect signs of antiphospholipid syndrome can make it difficult to diagnose the cause of the disease.
The symptoms of APS, depending on which organ is affected by thrombosis, will be as follows:
- An increase in blood pressure is observed with thrombosis of the renal arteries.
- When the pulmonary artery is blocked by a thrombus, PE develops, which leads to a sharp deterioration in a person's well-being. Sometimes the death of a patient can come instantly.
- Bleeding in the gastrointestinal tract.
- Spleen infarction.
- The appearance of subcutaneous hemorrhages, skin necrosis, ulcers on the legs - all these symptoms develop with damage to the dermis.
The AFS clinic is diverse. It is impossible to describe the exact symptoms, since any organs and systems can be involved in the pathological process.
APS treatment should be comprehensive. Its main focus is to prevent complications of thrombosis.
The patient must comply with the following recommendations:
- Refuse unbearable physical activity.
- You can't stay motionless for a long time.
- Refusal from traumatic sports.
- Refusal to fly.
Drug therapy is reduced to the appointment of the following drugs:
- Warfarin is a drug from the group of indirect anticoagulants.
- Heparin, Calcium nadroparin, Enoxaparin sodium - drugs related to direct anticoagulants.
- Aspirin, Dipyridamole, Pentoxifylline are antiplatelet drugs.
- If the patient is in serious condition, then he is injected with high doses of glucocorticosteroids, plasma transfusion is performed.
Taking anticoagulants and antiplatelet agents should be long-term. Sometimes these drugs are prescribed for life.
AFS is not a sentence. If the disease is diagnosed at the initial stage of its development, then the prognosis is favorable. In this case, the patient must follow all the doctor's recommendations, take the drugs that he prescribes. Women have a high chance of conceiving and having a healthy child.
The course of the disease is complicated by systemic lupus erythematosus, thrombocytopenia, persistent increase in blood pressure, high levels of antibodies to the cardiolipin antigen with a tendency to increase.
Without fail, a patient with a diagnosed APS should be observed by a rheumatologist. He will need to regularly donate blood for analysis, as well as undergo other diagnostic and therapeutic procedures.
Antiphospholipid antibodies in the blood - normal or pathological?
Sometimes the level of antiphospholipid antibodies can be elevated in a healthy person. In 12% of people, these antibodies are present in the blood, but they do not develop diseases. The older a person is, the higher the indicators of pathological immunoglobulins can be. There is also the likelihood of a false-positive Wasserman reaction, for which the patient should be prepared. The main thing is not to panic and undergo a comprehensive diagnosis.
Video: APS and other thrombophilia in obstetrics:
The author of the article: Volkov Dmitry Sergeevich | c. m. n. surgeon, phlebologist
Education: Moscow State University of Medicine and Dentistry (1996). In 2003 he received a diploma from the Educational and Scientific Medical Center of the Presidential Administration of the Russian Federation.