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What medications can cause secondary afs. Antiphospholipid syndrome (APS): essence, development, causes, diagnosis, treatment, why it is dangerous

Antiphospholipid syndrome (APS) or antiphospholipid antibody syndrome (SAFA) four decades ago was not known even to doctors not involved in this problem, not to mention patients. People started talking about it only in the early 80s of the last century, when the symptom complex was presented in detail by the London doctor Graham Hughes, so APS can also be found under this name - Hughes syndrome (some authors call it Hughes syndrome, which is probably also correct).

Why does this disease frighten doctors, patients and, especially, women who dream of motherhood? It's all about the action of antiphospholipid antibodies (APLA), which cause increased thrombus formation in the venous and arterial vessels of the circulatory system, which complicates the course of pregnancy, provokes miscarriages and premature birth, where the fetus often dies. In addition, it should be noted that the antiphospholipid antibody syndrome itself is more often detected in the female half of humanity who are of reproductive age (20–40 years). Men are luckier in this regard.

Basis of development of phospholipid antibody syndrome

The reason for the formation of this symptom complex is the appearance of antibodies (AT), the action of which is directed towards phospholipids that inhabit the membranes of various cells of many tissues of a living organism (blood plates - platelets, nerve cells, endothelial cells).

Phospholipids present on cell membranes and acting as antigens differ in their structure and ability to produce an immune response, and therefore are divided into types, for example, neutral and anionic (negatively charged) phospholipids - these two classes are the most common.

Thus, if there are different classes of phospholipids, then antibodies to them will represent a rather diverse community. Antiphospholipid antibodies (APLA) must be of different directions and have the ability to react with certain determinants (either anionic or neutral). The most famous, widespread, and of great clinical importance are immunoglobulins, which are used to diagnose APS:

Lupus anticoagulant(immunoglobulins of classes G or M - IgG, IgM) - this population was first discovered in patients suffering from SLE (systemic lupus erythematosus) and very prone to thrombosis;
Antibodies to cardiolipin antigen, which is the main component of the test for syphilis, the so-called Wassermann reaction. As a rule, these antibodies are immunoglobulins of classes A, G, M;
ATs that manifest themselves in the mixture cholesterol, cardiolipin, phosphatidylcholine (false-positive result of the Wasserman reaction);
Beta-2-glycoprotein-1-cofactor-dependent antibodies to phospholipids(total immunoglobulins of classes A, G, M). β-2-GP-1 itself is a natural anticoagulant, that is, a substance that prevents the unnecessary formation of blood clots. Naturally, the appearance of immunoglobulins to beta-2-GP-1 leads to thrombosis.

The study of antibodies to phospholipids is very important in diagnosing the syndrome, because in itself it is associated with certain difficulties.

Diagnosis of antiphospholipid syndrome

Of course, antiphospholipid syndrome can be suspected based on a number of clinical symptoms, but the final diagnosis must be established based on a combination of symptoms and an immunological examination of the patient, which implies a certain (and quite wide) list of laboratory tests. These include traditional methods: a general (with platelet count) and biochemical blood test, including a coagulogram, and specific tests aimed at identifying antibodies to phospholipids.

Insufficient examination (meaning the determination of one, often the most standardized and accessible method, which, for example, is often considered the anticardiolipin test), will most likely lead to overdiagnosis, because this analysis gives a positive result in other pathological conditions.

Very important laboratory diagnostic methods today are the determination of:

With antiphospholipid syndrome, various vessels can be affected: from capillaries to large arterial trunks located in any part of the human body, so the range of symptoms of this pathology is extremely wide. It affects various areas of medicine, thereby attracting many specialists: rheumatologists, neurologists, cardiologists, obstetricians, dermatologists, etc.

Thrombosis in veins and arteries

Most often, doctors are faced with thrombosis, which is recurrent and affects. The blood clots formed there, breaking off, are sent to the vessels of the lungs, blocking them, and this entails the occurrence of such a dangerous, and often fatal, condition, How . Here everything depends on the size of the incoming blood clot and the caliber of the vessel in which this blood clot is stuck. If the main trunk of the pulmonary artery (PA) is closed, then one cannot count on a favorable outcome - reflex cardiac arrest leads to instant death of a person. Cases of blockage of small branches of the pulmonary artery provide a chance for survival, but do not exclude hemorrhages, pulmonary hypertension, pulmonary infarction and the development of heart failure, which also does not “paint” particularly rosy prospects.

In second place in terms of frequency of occurrence can be thrombosis in the vessels of the kidneys and liver with the formation of corresponding syndromes (nephrotic,).

Although less common, thrombosis of the subclavian veins or retinal vessels occurs, as well as thrombosis localized in the central vein of the adrenal glands, which, after hemorrhages and necrosis, forms chronic adrenal insufficiency in the patient.

In other situations (depending on the location), thrombosis is among the triggers for the occurrence.

Arterial thrombosis causes ischemia with the development of necrosis. In short, heart attacks, aortic arch syndrome, gangrene, aseptic necrosis of the femoral head are all a consequence of arterial thrombosis.

APS during pregnancy is a difficult task in obstetric practice

Antiphospholipid antibody syndrome during pregnancy is on the list of particularly difficult tasks assigned to obstetrics, because a third of women who are anticipating the happiness of motherhood instead receive tears and disappointments. In general, we can say that obstetric pathology has incorporated the most characteristic, but rather dangerous features of antiphospholipid antibody syndrome:

Miscarriage that becomes habitual;
Recurrent spontaneous miscarriages (1st trimester), the risk of which increases in proportion to the increase in class G immunoglobulins to the cardiolipin antigen;
FPI (fetoplacental insufficiency), which creates conditions unsuitable for the normal formation of a new organism, resulting in a delay in its development, and often death in the womb;
with a risk of preeclampsia, eclampsia;
Chorea;
Thrombosis (both in veins and arteries), recurring again and again;
Hypertension in pregnancy;
Early onset and severe course of the disease;
hellp syndrome is a dangerous pathology of the 3rd trimester (35 weeks and beyond), an emergency condition in obstetric practice (rapid increase in symptoms: vomiting, epigastric pain, headache, swelling);
Early, untimely separation of the placenta;
Childbirth before 34 weeks;
Unsuccessful IVF attempts.

The development of pathological changes during pregnancy, of course, starts with vascular thrombosis, placental ischemia, and placental insufficiency.

Important - don't miss it!

Women with such pathology during gestation require special attention and dynamic monitoring. The doctor who is caring for her knows what the pregnant woman may face and what she risks, so she prescribes additional examinations:

at certain intervals in order to always see how the blood coagulation system behaves;
Ultrasound examination of the fetus with;
Ultrasound diagnostics of blood vessels of the head and neck, eyes, kidneys, lower extremities;
to avoid unwanted changes in the heart valves.

These measures are taken to prevent the development of thrombocytopenic purpura, hemolytic-uremic syndrome and, of course, such a formidable complication as. Or exclude them if the doctor has even the slightest doubt.

Of course, not only an obstetrician and gynecologist is involved in monitoring the development of pregnancy in women with antiphospholipid syndrome. Taking into account the fact that APS causes many organs to suffer, different specialists can be involved in the work: a rheumatologist - first of all, a cardiologist, a neurologist, etc.

Women with APS during the gestational period are advised to take glucocorticosteroids and antiplatelet agents (in small dosages prescribed by a doctor!). Immunoglobulins and heparin are also indicated, but they are used only under coagulation control.

But for girls and women who already know about “their APS” and are planning a pregnancy in the future, but are now thinking about “living for themselves,” I would like to remind you that oral contraceptives are not suitable for them, since they can do a bad job, so it’s better to try find another method of contraception.

The influence of AFLA on organs and systems

It is quite difficult to predict what can be expected from AFLA syndrome; it can create a dangerous situation in any organ. For example, does not remain aloof from unpleasant events in the body brain(GM). Thrombosis of its arterial vessels is the cause of diseases such as recurrent, which can have not only their characteristic symptoms (paresis and paralysis), but also be accompanied by:

Convulsive syndrome;
Dementia, steadily progressing and driving the patient’s brain into a “vegetative” state;
Various (and often very unpleasant) mental disorders.

In addition, other neurological symptoms can be encountered with antiphospholipid antibody syndrome:

Migraine-like headaches;
Erratic involuntary movements of the limbs, characteristic of chorea;
Pathological processes in the spinal cord, leading to motor, sensory and pelvic disorders, clinically coinciding with transverse myelitis.

Heart pathology, caused by the influence of antiphospholipid antibodies, can have not only severe symptoms, but also a serious prognosis regarding the preservation of health and life, because an emergency condition is myocardial infarction, is the result of thrombosis of the coronary arteries, however, if only the smallest branches are affected, then at first you can get by impaired contractility of the heart muscle. APS “takes an active part” in the formation of valve defects, in more rare cases it contributes formation of intraatrial thrombi and misdirects diagnostics as doctors begin to suspect cardiac myxoma.

APS can also cause a lot of trouble for other organs:

The variety of symptoms indicating damage to a particular organ often allows antiphospholipid syndrome to occur in different forms, such as pseudosyndromes simulating another pathology. It often behaves like vasculitis, sometimes it manifests itself as the onset of multiple sclerosis, in some cases doctors begin to suspect a heart tumor, in others – nephritis or hepatitis...

And a little about treatment...

The main goal of treatment is the prevention of thromboembolic complications. First of all, the patient is warned about the importance of compliance regime:

Do not lift heavy objects, physical activity is feasible, moderate;
Staying in a stationary position for a long time is unacceptable;
Sports activities, even with minimal risk of injury, are extremely undesirable;
Air travel for a long time is strongly not recommended; short trips should be agreed with your doctor.

Treatment with pharmaceuticals includes:

Treatment with antiplatelet agents and/or anticoagulants accompanies the patient for a long time, and some patients are forced to “sit” on them for the rest of their lives.

The prognosis for APS is not so bad if you follow all the doctors’ recommendations. Early diagnosis, constant prevention of relapses, timely treatment (with due responsibility on the part of the patient) give positive results and give hope for a long, high-quality life without exacerbations, as well as for a favorable course of pregnancy and a safe birth.

Difficulties in prognostic terms are presented by such unfavorable factors as the combination of ASP + SLE, thrombocytopenia, persistent arterial hypertension, and a rapid increase in antibody titers to the cardiolipin antigen. Here you can only sigh heavily: “The ways of the Lord are mysterious...”. But this does not mean that the patient has so little chance...

All patients with a specified diagnosis of “Antiphospholipid syndrome” are registered with a rheumatologist, who monitors the course of the process, periodically prescribes tests (, serological markers), carries out prevention and, if necessary, treatment.

Did you find antiphospholipid bodies in the analysis? Seriously, but don't panic...

In the blood of healthy people, the concentration of APLA usually does not show high results. At the same time, it cannot be said that they are not detected at all in this category of citizens. Up to 12% of people examined may have antibodies to phospholipids in their blood, but still not get sick. By the way, with age, the frequency of detection of these immunoglobulins will most likely increase, which is considered a completely natural phenomenon.

And yet, sometimes there are cases that make some especially impressionable people get pretty worried or even experience shock. For example, a person went for some kind of examination, which involved conducting many laboratory tests, including an analysis for syphilis. And the test turns out to be positive... Then, of course, they will recheck everything and explain that the reaction was false positive and, possibly, due to the presence of antiphospholipid antibodies in the blood serum. However, if this happens, then we can advise you not to panic prematurely, but also not to calm down completely, because antiphospholipid antibodies may someday remind you of themselves.

Video: lectures on APS

General information

About diagnosing APS

APS and other thrombophilias in obstetrics

What is antiphospholipid syndrome?

Antiphospholipid syndrome is an autoimmune disease accompanied by the development of thrombosis of arteries and veins, as well as complications during pregnancy, including fetal death.

Causes of antiphospholipid syndrome

Antiphospholipid syndrome is a relatively recently discovered disease, the causes of which are still under study. It can be primary, that is, not associated with other diseases, or secondary. The first variant of antiphospholipid syndrome occurs, as a rule, in individuals with a hereditary predisposition. In this case, the presence of the disease in other members of the patient’s family is typical, often in a latent form. The secondary variant of antiphospholipid syndrome is associated with other pathological conditions:

Antiphospholipid syndrome can also occur with long-term use of a number of medications, in particular oral contraceptives, quinidine and psychotropic drugs. Antiphospholipid syndrome is based on a violation of the immune system and the production of pathological proteins - autoantibodies that bind to the body's own phospholipids. This leads to an imbalance between the blood coagulation and anticoagulation systems. As a result of antiphospholipid syndrome, increased blood clotting occurs, which is accompanied by the development of various thromboses.

Signs and symptoms of antiphospholipid syndrome

Manifestations of antiphospholipid syndrome can be extremely diverse and range from mild clinically undetectable thrombosis to severe strokes and heart attacks at a young age. Patients with this disease can be detected accidentally during routine examinations due to false-positive Wasserman reactions to syphilis, which are characteristic of antiphospholipid syndrome. The first manifestations of the disease can be in the form of an accentuated vascular pattern on the body (livedo reticularis), especially on the legs, feet, hips, hands, point hemorrhages and repeated thrombosis of the veins of the lower extremities. With antiphospholipid syndrome, non-healing ulcers may develop on the legs, and if arterial thrombosis has occurred, gangrene of the toes may develop.

In the case of a severe course of the disease, sudden deterioration of vision (up to blindness) due to thrombosis of retinal vessels, kidney damage and aseptic necrosis of the knee and hip joints are characteristic. With antiphospholipid syndrome, myocardial infarctions and strokes are common, occurring at a young age (often before 40 years of age), and the development of arterial hypertension. But this pathology is most actively detected in patients of reproductive age who consult a doctor because of repeated pregnancy terminations, which are also a consequence of the disease. Today it is believed that antiphospholipid syndrome is the cause of approximately 20% of miscarriages. Complications of antiphospholipid syndrome include strokes and myocardial infarctions, thrombosis of the veins and arteries of the lower extremities and repeated pulmonary embolisms, spontaneous abortions during pregnancy, gangrene, blindness, arterial hypertension and chronic renal failure.

Diagnosis of antiphospholipid syndrome

Given the frequent subclinical course of antiphospholipid syndrome, it is necessary to use laboratory methods to confirm the diagnosis. All pregnant women with a burdened obstetric history must be examined for this disease.

The first laboratory methods used to make a diagnosis of antiphospholipid syndrome are a coagulation test (assessing clotting time and activated partial thromboplastin time) and coagulation tests with the addition of various snake venoms. In addition, a general blood test can detect a decrease in platelets. In laboratory conditions, the diagnosis of antiphospholipid syndrome is confirmed by the detection of anticardiolipin antibodies in the blood (most often antibodies to cardiolipin class G, lupus anticoagulant and b2-glycoprotein-1-cofactor-dependent antibodies). Considering that these autoantibodies can periodically disappear, to make a diagnosis of antiphospholipid syndrome, two tests for their presence are necessary with an interval of 6 weeks.

Instrumental diagnostic methods are used to determine the degree of damage to internal organs in antiphospholipid syndrome. For this purpose, ultrasound examination of the abdominal organs, chest radiography, echocardiography and magnetic resonance imaging of the brain are used. In the case of thrombosis of veins or arteries acutely developed as a result of antiphospholipid syndrome, the use of arteriography and duplex ultrasound examination of the vessels of the extremities is useful.

Differential diagnosis of antiphospholipid syndrome is carried out with autoimmune connective tissue diseases (systemic lupus erythematosus, rheumatoid arthritis and systemic scleroderma, Sjogren's syndrome), hereditary thrombophilias, autoimmune thrombocytopenic purpura and recurrent miscarriage due to other causes.

Treatment and prevention of antiphospholipid syndrome

Treatment of antiphospholipid syndrome in the absence of complications is carried out on an outpatient basis, and often several specialists are involved in the treatment of the disease: therapists, cardiologists, hematologists, vascular surgeons and obstetricians-gynecologists. A patient with antiphospholipid syndrome may be hospitalized for selection of antithrombotic therapy if bleeding disorders increase, as well as if there is a threat of developing complications of the disease. Women with complicated pregnancy are required to be hospitalized.

If antiphospholipid syndrome is a consequence of other diseases or taking any medications, its cause should be eliminated first. Drug therapy consists of prescribing antithrombotic drugs - antiplatelet agents (dipyridamole, aspirin and pentoxifylline) and anticoagulants (heparin or its low molecular weight analogues - nadroparin, enoxaparin and dalteparin). These drugs reduce blood clotting and prevent the development of thrombosis. For antiphospholipid syndrome against the background of other autoimmune manifestations, glucocorticosteroids (prednisolone and methylprednisolone) can be prescribed. This group of drugs is especially often used to prevent complications of the disease during pregnancy. Extracorporeal detoxification methods (plasmapheresis) can have a certain effect in the treatment of antiphospholipid syndrome. It should be remembered that the tactics of treating the disease, as well as the use of any medications, are possible only after prior consultation with a doctor.

When severe thrombosis develops against the background of antiphospholipid syndrome, surgical interventions are sometimes resorted to, consisting of mechanical removal of the thrombus from the affected vein and artery or the formation of bypass blood flow paths.

Prevention of antiphospholipid syndrome consists, first of all, in timely examination and treatment of the disease at the slightest suspicion of its presence, preferably before pregnancy. It is not recommended to abuse long-term use of oral contraceptives. All women of reproductive age who have relatives who suffer from increased blood clotting and recurrent thrombosis should be examined for antiphospholipid syndrome.

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Antiphospholipid syndrome is a condition in which a person's immune system mistakenly produces antibodies against normal proteins found in the blood.

Antiphospholipid syndrome can lead to blood clots in large blood vessels, as well as pregnancy complications such as stillbirths and miscarriages.

Antiphospholipid syndrome is often complicated by thrombosis of the veins of the lower extremities. Blood clots can form in vital organs, including the heart, kidneys and lungs. Symptoms of the disease depend on the location of the blood clot. For example, thrombosis of a cerebral vessel causes a stroke with all its characteristic symptoms. There is no radical cure for antiphospholipid syndrome, but there are many medications that reduce the risk of blood clots in patients.

Causes of the disease

In antiphospholipid syndrome, blood proteins that bind phospholipids, a special type of substance that plays a key role in blood clotting (coagulation), become victims of the immune system. The immune system mistakenly perceives these proteins as foreign and produces antibodies against them. Antibodies are specialized proteins that are directed only against “harmful” molecules, like the cellular components of bacteria. When the immune system mistakenly attacks phospholipid-binding proteins, blood clots begin to form in the patient's blood.

There are two types of antiphospholipid syndrome:

Primary. If the patient does not have other autoimmune diseases, such as lupus, then this antiphospholipid syndrome is considered primary.
Secondary. If antiphospholipid syndrome develops against the background of another autoimmune disease, then it is considered secondary. In the case of a secondary syndrome, its cause is considered to be an initial autoimmune disease.

The cause of primary antiphospholipid syndrome is unknown, but there are several factors that play an important role in its occurrence:

Infections. People with AIDS, syphilis, hepatitis C and malaria are more likely to have antiphospholipid syndrome.
Medicines. Taking certain hypertension medications (hydralazine), anticonvulsants (phenytoin), and the antibiotic amoxicillin may increase your risk of the disease.
Genetic predisposition. Antiphospholipid syndrome is not considered a hereditary disorder, but studies show an increased risk of the disease in some families.

Risk factors

Currently known risk factors for antiphospholipid syndrome include:

Autoimmune diseases such as systemic lupus erythematosus (SLE) or Sjögren's disease.
The presence of certain infections such as syphilis and hepatitis C.
Taking a number of medications, including hydralazine, quinidine, phenytoin, amoxicillin and others.
Complicated family history.

According to statistics, antiphospholipid syndrome most often occurs in young and middle-aged women, although it can affect people of any gender and age. It is worth mentioning separately the risk of developing the symptoms themselves. It is quite possible for antiphospholipid syndrome to occur without any manifestations. Antiphospholipid antibodies may be elevated in the patient's blood, but blood clots do not form and there are no complaints.

The risk of blood clots increases in the following cases:

Pregnancy.
Temporary immobilization.
Surgical operations.
Smoking cigarettes.
High blood pressure.
Increased cholesterol levels.
Taking hormonal contraceptives.

Manifestations of antiphospholipid syndrome

The main signs of antiphospholipid syndrome may include:

Formation of blood clots in the veins of the lower extremities, which can break off and travel to the lungs (pulmonary embolism).
Repeated miscarriages or stillbirths. Other pregnancy complications, such as premature birth and preeclampsia, are possible.
Blockage of a cerebral vessel (stroke).

Other, rarer signs of antiphospholipid syndrome include:

Neurological symptoms. Chronic headaches, dementia and seizures are possible as a result of disruption of the blood supply to certain areas of the brain.
Rash. Some people develop a red, lace-like or mesh-shaped rash on their wrists and knees.
Cardiovascular diseases. Problems with the heart valves are common among patients with antiphospholipid syndrome. Normally, heart valves open and close to allow blood to flow in only one direction. When diseased, the valve may lose its function, causing blood to flow in the opposite direction (regurgitation). This leads to severe circulatory problems.
Bleeding. In some patients, the number of platelets, blood cells necessary for normal clotting, drops. With this condition, called thrombocytopenia, there may be no complaints. But if the number of platelets decreases too sharply, bleeding is possible, including from the nose and gums. Small red spots (petechiae) may form under the skin.

Rare signs of antiphospholipid syndrome:

Movement disorders in which the trunk and limbs twitch chaotically (chorea).
Problems with intelligence and memory.
Mental disorders such as depression or psychosis.
Sudden hearing loss.

When should you see a doctor?

If you have already been diagnosed with any autoimmune disease, be sure to talk to your doctor about an antiphospholipid antibody test.

Other reasons to urgently consult a doctor:

Pain and swelling in an arm or leg. Special attention is required if your veins are red and swollen. If you experience severe pain and swelling accompanied by shortness of breath, help is required immediately! This may indicate thrombosis of the veins of the extremities and a blood clot entering the lung.
Signs of a stroke. They can be varied, including numbness of the limbs, weakness, paralysis, difficulty speaking and understanding speech, visual disturbances, severe headache, dizziness, etc.
Vaginal bleeding during the first 20 weeks of pregnancy. Heavy bleeding or spotting may be a sign of miscarriage. If you have had repeated miscarriages and other pregnancy complications, then be especially attentive to your condition.

If you suffer from antiphospholipid syndrome and are trying to get pregnant, you need to undergo special treatment that will prevent miscarriage. Make sure your doctor is aware of your diagnosis and is taking any necessary steps.

Diagnosis of the disease

If the patient has had one or more episodes of thrombosis or unexplained miscarriage, the doctor may order blood tests to check clotting and the presence of antiphospholipid antibodies.

Blood tests to confirm antiphospholipid syndrome look for at least one of three antibodies:

Anti-cardiolipin.
Lupus anticoagulant.
Beta 2-glycoprotein I (B2GPI).

To confirm the diagnosis, antibodies must be detected in the blood at least twice, in tests performed at least 12 weeks apart.

Treatment of antiphospholipid syndrome

Doctors primarily prescribe medications that reduce the tendency of blood clots.

The standard initial treatment for antiphospholipid syndrome in the West consists of several anticoagulants:

Heparin. This drug is given intravenously, usually in combination with other anticoagulants such as warfarin (Coumadin).
Warfarin. After several days of combination therapy with heparin and warfarin, the doctor may stop the first drug and continue giving the patient warfarin. The patient sometimes needs to take warfarin tablets for the rest of his life.
Aspirin (acetylsalicylic acid). In some cases, the effect of the first two drugs is not enough, then the doctor additionally prescribes Aspirin in low doses. This treatment can also be very long-term.

Treatment of antiphospholipid syndrome during pregnancy is quite complex, expensive, requires regular injections and is associated with many risks. Some drugs, such as warfarin, cause birth defects during pregnancy and should not be used.

Heparin. Some forms of heparin - enoxaparin (Lovenox) and dalteparin (Fragmin) - are known as low molecular weight forms of heparin, which the patient can inject herself under the skin. Heparin is considered safe during pregnancy.
Aspirin. Along with low molecular weight heparin, the doctor may prescribe Aspirin in tablet form, which increases the chances of successfully bearing a child.

In rare cases, a pregnant woman may still be prescribed warfarin, but only when the benefit to the mother outweighs the risk to the fetus. Warfarin does not always cause birth defects in a baby, but the risk cannot be ignored. Treatment for antiphospholipid syndrome in pregnancy can be difficult, but miscarriage and other problems are usually prevented. If you are taking anticoagulants, your doctor will do regular blood tests to make sure the drugs do not interfere with clotting. If the dose of drugs is high for you, it may cause bleeding at the slightest injury. Therefore, constant monitoring is very important.

Depending on your treatment plan, there are several additional steps you can take to protect your health. While taking anticoagulants, there may be a tendency to bleeding. Bleeding can occur with a minor sports injury, and even after an accidental cut while shaving.

During anticoagulant therapy, follow these rules:

Avoid contact sports and other dangerous activities.
Use only a soft toothbrush to avoid damaging your gums.
Shave only with an electric razor to avoid cutting your skin.
Be especially careful with knives, scissors and other sharp objects. Automate kitchen and office work as much as possible so you have to deal with these items less often.

When using anticoagulants, you need to remember that they cannot be combined with many medications and dietary supplements.

Consult your doctor if you use:

Foods rich in vitamin K. This vitamin promotes blood clotting and reduces the effectiveness of anticoagulants. It is very important to understand how much vitamin K you consume daily. The average daily value for an adult man is 120 mcg, and for women - 90 mcg. If you eat small amounts of foods rich in vitamin K, this is not a problem. But if you are fond of spinach, Brussels sprouts, parsley and some other vegetables, this may interfere with the treatment.
Liquor and cranberry. On the other hand, cranberry juice and alcohol can dangerously increase the effects of warfarin. This may cause serious bleeding, so avoid these foods during treatment.
Over-the-counter drugs and dietary supplements. Many of these, including OTC analgesics, cold remedies, multivitamins, garlic capsules, ginkgo, and green tea products, do not combine with warfarin.

If you suffer from antiphospholipid syndrome and are not taking anticoagulants, you should follow several rules:

Be sure to tell all your doctors about your diagnosis.
Talk to your doctor about thrombosis prevention measures if you lie still for long periods of time (due to illness or surgery).
Don't smoke. If you have smoked before, be sure to quit the habit.
Reducing your blood cholesterol levels is an important step for preventing heart attack and stroke.

Possible complications

Complications of antiphospholipid syndrome depend on the organ in which the clot has formed. In severe cases, thrombosis can lead to vital organ failure and death.

Complications include:

Kidney failure. This condition develops due to insufficient blood flow to the kidneys.
Stroke. Insufficient blood supply to the brain can lead to irreversible damage to nerve structures, paralysis, and impairment of intelligence and behavior.
Cardiovascular problems. If a blood clot forms in the leg, it damages the valves in the veins that normally drain blood toward the heart. The veins dilate, the valves cannot perform their functions, the vessels become overfilled, and the blood stagnates in them. The result is chronic venous insufficiency, accompanied by swelling and discoloration of the limbs. Another possible complication is heart damage.
Lung problems. Pulmonary complications include high blood pressure in the lungs (pulmonary hypertension) and pulmonary embolism from a blood clot.
Complications of pregnancy. These include miscarriage, stillbirth, preterm birth and preeclampsia.

Antiphospholipid syndrome is a disease that includes a whole complex of symptoms related to a disorder of phospholipid metabolism. The essence of the pathology is that the human body mistakes phospholipids for foreign bodies, against which it produces specific antibodies.

What factor causes the formation of such a disease in women, men and children remains unknown today. Nevertheless, clinicians identify several predisposing sources, including infectious processes of a viral or bacterial nature.

Antiphospholipid syndrome corresponds to a large number of very diverse manifestations, including increased blood tone, skin damage, the formation of blood clots, etc.

To make a correct diagnosis, a wide range of laboratory tests are required, which must be supplemented by instrumental procedures and a thorough examination by a clinician.

Treatment of the pathology is based on conservative methods, but if it is severe, a procedure such as plasmapheresis may be necessary.

In the International Classification of Diseases, this syndrome does not have a separate code, but it belongs to the category “other coagulation disorders,” which is why the ICD-10 code will be D 68.0.

Etiology

The reasons against which phospholipid syndromes develop remain unknown, however, experts in the field of hematology and rheumatology note the presence of several predisposing factors.

Thus, men, women and children are susceptible to the formation of a similar disease against the background of:

genetic predisposition - the risk of developing signs of a similar disease increases significantly when a similar disease is diagnosed in close relatives;
and other rheumatological pathologies;
formation of oncological tumors, regardless of their location and the number of metastases;
the course of certain ailments affecting the central nervous system;
staphylococcal, streptococcal and a wide range of other bacterial infectious processes;
, and other autoimmune processes;
type C and B;
pathologies that provoke;
and other immunodeficiency conditions;
uncontrolled use of certain groups of medications, in particular interferons, oral contraceptives and psychotropic substances.

Antiphospholipid syndrome is extremely dangerous for pregnant females. It is in this category of patients that the development of complications is most often observed, not only with the course of the period of bearing a child, but also with the functioning of some internal organs.

It is not possible to establish the exact degree of occurrence of the pathology, but it is known that in 4% of cases completely healthy people are exposed to antiphospholipid syndromes. It is noteworthy that in females, antibodies to phospholipids are detected during laboratory diagnostics several times more often than in males. Moreover, clinicians have found that the older a person is, the more often such a deviation is detected, which is why it develops extremely rarely in a child.

Classification

There are several main types of this disease:

primary antiphospholipid syndrome- characterized by development in the absence of a particular disease. Its causes remain unclear, but it is believed to be influenced by family history, low-grade infections and drug overdose;
secondary API- differs in that it arises due to the occurrence in the human body of any pathological process of an autoimmune, oncological, rheumatic, infectious or medicinal nature.

Depending on the clinical manifestations, the following special forms of the disease are distinguished:

catastrophic APS- is expressed in a rapid course, the development of failure of all systems and internal organs, which is caused by the formation of blood clots of both large and small sizes;
APS in combination with vasculitis- in such situations, inflammatory processes occur in the vessels;
hypothrombinemia syndrome- with this variant of the course, there is an insufficient amount of thrombin in the blood. This substance takes part in the process of its coagulation and the formation of a blood clot;
microangiopathic syndromes- in turn, are divided into hemolytic-uremic syndrome, thrombotic or thrombocytopenic purpura and HELLP syndrome;
disseminated vascular coagulation- in addition to disruption of the blood coagulation system and the appearance of blood clots, hemorrhages develop.

Clinical criteria for antiphospholipid syndrome are not the main factors that make up the classification of the pathology. There is also a group of laboratory criteria that divides the API into:

seropositive- the main types of antibodies to phospholipids are detected in the patient through a wide range of laboratory blood tests;
seronegative- antibodies are not detected in the patient’s blood test.

Symptoms

Antiphospholipid syndromes consist of a wide variety of clinical manifestations, which will differ depending on the affected segment.

The very first and most common sign of the disease is the formation of blood clots, which can be venous (occur several times more often) and arterial. The veins most often involved in the pathology are the veins of the legs, liver, kidneys and retina, as well as the cerebral arteries.

Diagnostics

Due to the fact that the disease has pronounced clinical manifestations and also has specific laboratory abnormalities, there are no problems in establishing the correct diagnosis. However, to clarify it, instrumental examinations and a number of manipulations performed directly by a hematologist are required.

Thus, primary diagnostic measures include:

studying the medical history of not only the patient, but also his close relatives - to identify the most appropriate predisposing factor for a particular person;
collection and analysis of life history - this should also include information about the course of pregnancy;
a thorough physical examination, including palpation of the abdomen, examination of the extremities, assessment of visual acuity and skin condition, as well as listening to the patient using a phonendoscope and measuring blood tone;
a detailed survey of the patient - to determine the severity of symptoms, which will indicate the variant of the disease.

Laboratory diagnostics include:

general clinical blood test;
coagulogram - to assess blood clotting;
Coombs test;
enzyme immunoassay blood test;
serological tests;
blood biochemistry.

Instrumental diagnosis of antiphospholipid syndrome is aimed at:

Dopplerography of blood vessels;
Fetal ultrasound;
ECG and EchoCG;
Cardiography;
radiography of the peritoneum;
Doppler ultrasound of the arteries and veins of the legs, vessels of the kidneys, liver and head.

In addition, you may need consultation and examination with the following specialists:

gastroenterologist;
cardiologist;
obstetrician-gynecologist;
nephrologist;
pediatrician;
nephrologist;
therapist;
rheumatologist.

Treatment

Despite the fact that the clinical picture of APS has a negative impact on many internal organs and systems of the human body, therapy for the disease consists of using conservative techniques, which are also aimed at preventing the development of complications.

Drug treatment includes taking:

direct and indirect anticoagulants;
glucocorticoids - in case of catastrophic APS;
antiplatelet agents;
antibacterial agents.

In cases of severe antiphospholipid syndrome in men, women and children, the following is indicated:

intravenous administration of immunoglobulin;
performing plasmapheresis;
transfusion of fresh frozen plasma.

In addition, treatment should include:

performing moderate physical activity;
refusal to remain immobile for long periods of time and engage in active sports;
avoiding air travel;
exclusion of the use of oral contraceptives.

Other methods of therapy, in particular traditional medicine, are not used for antiphospholipid syndrome.

Possible complications

Late diagnosis of antiphospholipid syndrome, ignoring clinical signs and inadequate therapy leads to the formation of a large number of complications, including:

For pregnant females, the pathology is fraught with:

intrauterine fetal death;
miscarriages;
premature birth;
undeveloped pregnancy;
hemolytic disease of the fetus;
intrauterine fetal hypoxia.

Prevention and prognosis

Given that the exact causes of the disease remain unknown, preventive clinical recommendations are aimed at following general rules:

maintaining a healthy and moderately active lifestyle;
using only those medications prescribed by the clinician;
timely treatment of bacterial and viral infectious processes, as well as other ailments that can cause the appearance of APS;
Regular visits to an obstetrician-gynecologist are indicated for pregnant women.

In addition, do not forget about preventive examinations at a medical institution and blood tests at least twice a year.