Antiphospholipid syndrome - what is it. Diagnostics, analyzes and clinical guidelines for atf syndrome

Phospholipid syndrome is a relatively common pathology of autoimmune origin. Against the background of the disease, lesions of blood vessels, kidneys, bones and other organs are often observed. In the absence of therapy, the disease can lead to dangerous complications up to the death of the patient. Moreover, the disease is often found in women during pregnancy, which endangers the health of the mother and child.

Of course, many people seek additional information by asking questions about the causes of the disease. What symptoms should you look out for? Is there a phospholipid syndrome test? Can medicine offer effective treatments?

Phospholipid syndrome: what is it?

This disease was first described not so long ago. Official information about him was published in the 1980s. Since the English rheumatologist Graham Hughes worked on the study, the disease is often called Hughes's syndrome. There are other names - antiphospholipid syndrome and syndrome

Phospholipid Syndrome is an autoimmune disease in which the immune system starts producing antibodies that attack the body's own phospholipids. Since these substances are part of the membrane walls of many cells, the lesions with such an ailment are significant:

• Antibodies attack healthy endothelial cells, reducing the synthesis of growth factors and prostacyclin, which is responsible for vascular wall dilation. Against the background of the disease, there is a violation of platelet aggregation.
• Phospholipids are also contained in the walls of the platelets themselves, which leads to increased aggregation, as well as rapid destruction.
• In the presence of antibodies, a weakening of heparin activity is also observed.
• The destruction process does not bypass nerve cells either.

Blood begins to coagulate in the vessels, forming blood clots, which disrupt the blood flow, and therefore the functions of various organs - this is how phospholipid syndrome develops. The causes and symptoms of this ailment are of interest to many people. After all, the sooner the disease is detected, the fewer complications the patient will develop.

The main reasons for the development of the disease

Why do people develop phospholipid syndrome? The reasons may vary. It is known that quite often patients have a genetic predisposition. The disease develops in case of malfunctioning of the immune system, which for one reason or another begins to produce antibodies to the cells of its own body. In any case, the disease must be provoked by something. To date, scientists have managed to identify several risk factors:

• Often, phospholipid syndrome develops against the background of microangiopathies, in particular, trobocytopenia, hemolytic-uremic syndrome.
• Risk factors include other autoimmune diseases, such as lupus erythematosus, vasculitis, scleroderma.
• The disease often develops in the presence of malignant tumors in the patient's body.
• Risk factors include infectious diseases. Infectious mononucleosis and AIDS are especially dangerous.
• Antibodies can appear with disseminated intravascular coagulation.
• It is known that the disease can develop while taking certain medications, including hormonal contraceptives, psychotropic drugs, Novocainamide, etc.

Naturally, it is important to find out exactly why the patient developed phospholipid syndrome. Diagnostics and treatment should identify and, if possible, eliminate the root cause of the disease.

Damage to the cardiovascular system in phospholipid syndrome

Blood and vessels are the first "targets" that phospholipid syndrome strikes. Its symptoms depend on the stage of development of the disease. Blood clots, as a rule, first form in the small vessels of the extremities. They disrupt blood flow, which is accompanied by tissue ischemia. The affected limb is always colder to the touch, the skin turns pale, and the muscles gradually atrophy. Prolonged tissue malnutrition leads to necrosis and subsequent gangrene.

Deep vein thrombosis of the extremities is also possible, which is accompanied by the appearance of edema, pain, and impaired mobility. Phospholipid syndrome can be complicated by thrombophlebitis (inflammation of the vascular walls), which is accompanied by fever, chills, redness of the skin in the affected area, and acute, sharp pain.

The formation of blood clots in large vessels can lead to the development of the following pathologies:

• aortic syndrome (accompanied by a sharp increase in pressure in the vessels of the upper body);
• syndrome (this condition is characterized by swelling, cyanosis of the skin, bleeding from the nose, trachea and esophagus);
• (accompanied by impaired blood circulation in the lower body, swelling of the limbs, pain in the legs, buttocks, abdominal cavity and groin).

Thrombosis also affects the work of the heart. Often the ailment is accompanied by the development of angina pectoris, persistent arterial hypertension, and myocardial infarction.

Kidney damage and main symptoms

The formation of blood clots leads to impaired blood circulation not only in the limbs - internal organs, in particular the kidneys, also suffer. With prolonged development of phospholipid syndrome, the so-called kidney infarction is possible. This condition is accompanied by back pain, a decrease in the amount of urine and the presence of blood impurities in it.

A blood clot can block the renal artery, which is accompanied by severe pain, nausea, and vomiting. This is a dangerous condition - if untreated, a necrotic process may develop. The dangerous consequences of phospholipid syndrome include renal microangiopathy, in which small blood clots form directly in the renal glomeruli. This condition often leads to the development of chronic renal failure.

Sometimes there is a violation of blood circulation in the adrenal glands, which leads to a violation of the hormonal background.

What other organs can be affected?

Phospholipid syndrome is an ailment that affects many organs. As already mentioned, antibodies affect the membranes of nerve cells, which cannot be without consequences. Many patients complain of persistent severe headaches, which are often accompanied by dizziness, nausea and vomiting. There is a possibility of developing various mental disorders.

In some patients, blood clots are found in the vessels that supply the visual analyzer. Prolonged oxygen and nutrient deficiency leads to optic atrophy. Thrombosis of the retinal vessels is possible with subsequent hemorrhage. Some of the eye pathologies, unfortunately, are irreversible: the visual impairment remains with the patient for life.

Bones may also be involved in the pathological process. Reversible osteoporosis is often diagnosed in humans, with skeletal deformities and frequent fractures. More dangerous is aseptic bone necrosis.

Skin lesions are also characteristic of the disease. Spider veins are often formed on the skin of the upper and lower extremities. Sometimes you notice a very characteristic rash that looks like small, punctate hemorrhages. Some patients develop erythema on the soles of the feet and palms. There is a frequent formation of subcutaneous hematomas (for no apparent reason) and hemorrhage under the nail plate. Long-term violation of tissue trophism entails the appearance of ulcers, which take a long time to heal and are difficult to treat.

We have found out what constitutes phospholipid syndrome. The causes and symptoms of the disease are extremely important questions. After all, the treatment regimen chosen by the doctor will depend on these factors.

Phospholipid syndrome: diagnosis

Of course, in this case, it is extremely important to detect the presence of the disease in time. The doctor may suspect phospholipid syndrome even during anamnesis. The presence of thrombosis and trophic ulcers, frequent miscarriages, and signs of anemia in a patient can prompt this idea. Of course, in the future, additional examinations are carried out.

The analysis for phospholipid syndrome consists in determining the level of antibodies to phospholipids in the blood of patients. In the general analysis of blood, you can notice a decrease in the level of platelets, an increase in ESR, an increase in the number of leukocytes. Often, the syndrome is accompanied by hemolytic anemia, which can also be seen during laboratory tests.

Additionally, blood is carried out. In patients, an increase in the amount of gamma globulins is observed. If the liver was damaged against the background of pathology, then the amount of bilirubin and alkaline phosphatase in the blood increases. In the presence of kidney disease, an increase in creatinine and urea levels can be observed.

For some patients, specific immunological blood tests are also recommended. For example, laboratory tests can be done to determine rheumatoid factor and lupus coagulant. With phospholipid syndrome in the blood, you can detect the presence of antibodies to erythrocytes, an increase in the level of lymphocytes. If there are suspicions of severe damage to the liver, kidneys, bones, then instrumental examinations are carried out, including an X-ray, ultrasound, tomography.

What are the complications of the disease?

If untreated, phospholipid syndrome can lead to extremely dangerous complications. Against the background of the disease, blood clots form in the vessels, which is already dangerous in itself. Blood clots clog the vessels, disrupting normal blood circulation - tissues and organs do not receive enough nutrients and oxygen.

Often, against the background of the disease, patients develop a stroke and myocardial infarction. Blockage of the vessels of the extremities can lead to the development of gangrene. As mentioned above, patients have impaired renal and adrenal function. The most dangerous consequence is pulmonary embolism - this pathology develops acutely, and not in all cases the patient can be delivered to the hospital on time.

Pregnancy in patients with phospholipid syndrome

As already mentioned, the phospholipid syndrome is diagnosed during pregnancy. What is the danger of the disease and what to do in a similar situation?

Due to the phospholipid syndrome, blood clots form in the vessels, which clog the arteries that carry blood to the placenta. The embryo does not receive enough oxygen and nutrients, in 95% of cases this leads to miscarriage. Even if the pregnancy is not interrupted, there is a risk of early placental abruption and the development of late gestosis, which is very dangerous for both the mother and the child.

Ideally, a woman should be tested at the planning stage. However, phospholipid syndrome is often diagnosed during pregnancy. In such cases, it is very important to notice the presence of an ailment in time and take the necessary measures. Anticoagulants in small doses may be prescribed for the expectant mother. In addition, a woman should undergo regular examinations so that the doctor can notice the onset of placental abruption in time. Every few months, expectant mothers undergo a course of general strengthening therapy, taking medications containing vitamins, minerals and antioxidants. With the right approach, pregnancies often end well.

What does the treatment look like?

What if a person has phospholipid syndrome? Treatment in this case is complex, and it depends on whether the patient has certain complications. Since blood clots form against the background of the disease, therapy is primarily aimed at thinning the blood. The treatment regimen usually includes the use of several groups of drugs:

• First of all, indirect anticoagulants and antiplatelet agents are prescribed ("Aspirin", "Warfarin").
• Often therapy includes selective anti-inflammatory drugs of non-steroidal origin, in particular "Nimesulide" or "Celecoxib".
• If the disease is associated with systemic lupus erythematosus and some other autoimmune diseases, your doctor may prescribe glucocorticoids (hormonal anti-inflammatory drugs). Along with this, immunosuppressive drugs can be used that suppress the activity of the immune system and reduce the production of dangerous antibodies.
• Immunoglobulin is sometimes given intravenously to pregnant women.
• Patients periodically take medications containing B vitamins.
• For general recovery, protection of blood vessels and cell membranes, antioxidant drugs are used, as well as drugs that contain a complex of polyunsaturated fatty acids (Omacor, Mexicor).

Electrophoresis procedures are beneficial for the patient's condition. When it comes to secondary phospholipid syndrome, it is important to control the primary disease. For example, patients with vasculitis and lupus should receive adequate treatment for these particular pathologies. It is also important to detect infectious diseases in time and carry out appropriate therapy until complete recovery (if possible).

Patient predictions

If phospholipid syndrome was diagnosed on time and the patient received the necessary help, then the prognosis is very favorable. Unfortunately, it is impossible to get rid of the disease forever, but with the help of medications it is possible to control its exacerbations and carry out preventive treatment of thrombosis. Situations in which the disease is associated with thrombocytopenia and high blood pressure are considered dangerous.

In any case, all patients diagnosed with phospholipid syndrome should be under the supervision of a rheumatologist. How many times the analysis is repeated, how often you need to undergo examinations with other doctors, what drugs you need to take, how to monitor the state of your own body - the attending physician will tell you about all this.

Content

Autoimmune diseases are difficult to successfully treat, since immune cells come into conflict with certain vital structures of the body. Among the common health problems is phospholipid syndrome, when the immune system perceives the structural component of the bone as a foreign body, trying to destroy it.

What is antiphospholipid syndrome

Any treatment should begin with a diagnosis. Antiphospholipid syndrome is an autoimmune pathology with a persistent resistance of immunity to phospholipids. Since these are irreplaceable structures for the formation and strengthening of the skeletal system, improper actions of the immune system can negatively affect the health and vital activity of the whole organism. If antiphospholipid antibodies are observed in the blood, the disease does not proceed alone, it is accompanied by venous thrombosis, myocardial infarction, stroke, chronic non-carrying of pregnancy.

This disease may predominate in its primary form, i.e. develops independently, as a single ailment of the body. The antiphospholipid syndrome also has a secondary form (VAFS), i.e. becomes a complication of another chronic disease of the body. Alternatively, it can be Budd-Chiari syndrome (thrombosis of the hepatic veins), superior vena cava syndrome, and other pathogenic factors.

Antiphospholipid syndrome in men

Extensive medical practice describes cases of illness in the stronger sex, although these are much less common. Antiphospholipid syndrome in men is represented by blockage of the lumen of the veins, as a result of which the systemic blood flow in certain internal organs and systems is disturbed. Insufficient blood supply can lead to serious health problems such as:

• pulmonary embolism;
• pulmonary hypertension;
• episodes of PE;
• thrombosis of the central vein of the adrenal glands;
• gradual death of the lung, hepatic tissue, liver parenchyma;
• arterial thrombosis, disorders of the central nervous system are not excluded.

Antiphospholipid syndrome in women

The disease entails catastrophic consequences, so doctors insist on immediate diagnosis and effective treatment. In most clinical pictures, the patient is the fairer sex, and not always pregnant. Antiphospholipid syndrome in women is the cause of diagnosed infertility, and the results of an APS examination show that a huge number of blood clots are concentrated in the blood. The international code ICD 10 includes the indicated diagnosis, which more often progresses during pregnancy.

Antiphospholipid syndrome in pregnant women

During pregnancy, the danger lies in the fact that during the formation of blood vessels of the placenta, thrombosis develops and rapidly progresses, which disrupts the blood supply to the fetus. The blood is not enriched with sufficient oxygen, and the embryo suffers from oxygen starvation, does not receive nutrients valuable for intrauterine development. You can determine the ailment at a routine screening.

If antiphospholipid syndrome develops in pregnant women, for expectant mothers it is fraught with premature and pathological childbirth, early miscarriage, feto-placental insufficiency, late gestosis, placental abruption, congenital diseases of newborns. APS during pregnancy is a dangerous pathology at any obstetric stage that can result in diagnosed infertility.

Causes of antiphospholipid syndrome

It is difficult to determine the etiology of the pathological process, and modern scientists to this day are lost in conjecture. It has been established that Sneddon's syndrome (also called antiphospholipid syndrome) may have a genetic predisposition in the presence of loci DR7, DRw53, HLA DR4. In addition, the development of the disease against the background of infectious processes in the body is possible. Other causes of antiphospholipid syndrome are detailed below:

• autoimmune diseases;
• long-term use of medications;
• oncological diseases;
• pathological pregnancy;
• pathology of the cardiovascular system.

Antiphospholipid Syndrome Symptoms

It is possible to determine the disease by a blood test, but in addition a number of laboratory tests are to be carried out to detect the antigen. Normally, it should not be in the biological fluid, and its appearance only indicates that the body is fighting its own phospholipids. The main symptoms of antiphospholipid syndrome are detailed below:

• aPS diagnostics by vascular pattern on sensitive skin;
• convulsive syndrome;
• severe migraine attacks;
• deep vein thrombosis;
• mental disorders;
• thrombosis of the lower extremities;
• decreased visual acuity;
• superficial vein thrombosis;
• adrenal insufficiency;
• retinal vein thrombosis;
• ischemic neuropathy of the optic nerve;
• hepatic portal vein thrombosis;
• sensorineural hearing loss;
• acute coagulopathy;
• recurrent hyperkinesis;
• dementia syndrome;
• transverse myelitis;
• thrombosis of the cerebral arteries.

Diagnostics of the antiphospholipid syndrome

To determine the pathogenesis of the disease, it is necessary to undergo an examination for APS, in which it is required to take a blood test for serological markers - lupus anticoagulant and antibodies Ab to cardiolipin. Diagnosis of antiphospholipid syndrome, in addition to taking tests, provides an anticardiolipin test, AFL, coagulogram, Doppler, CTG. The diagnosis is based on blood counts. To increase the reliability of the results, on the recommendation of the attending physician, an integrated approach to the problem is shown. So, pay attention to the following symptom complex:

• lupus anticoagulant increases the number of thrombosis, while he himself was first diagnosed with systemic lupus erythematosus;
• antibodies to cardiolipin resist natural phospholipids, promote their rapid destruction;
• antibodies in contact with cardiolipin, cholesterol, phosphatidylcholine are determined by a false positive Wasserman reaction;
• beta2-glycoprotein-1-cofactor-dependent antiphospholipid antibodies become the main cause of thrombotic symptoms;
• antibodies to beta-2-glycoprotein, limiting the patient's chances of getting pregnant safely.
• AFL-negative subtype without detection of antibodies to phospholipids.

Treatment of antiphospholipid syndrome

If APS or VAFS is diagnosed, and the signs of the disease are clearly expressed without additional clinical examinations, this means that treatment must be started in a timely manner. The approach to the problem is complex, it includes taking medications of several pharmacological groups. The main goal is to normalize the systemic circulation, to prevent the formation of blood clots with subsequent congestion of the body. So, the main treatment for antiphospholipid syndrome is presented below:

1. Glucocorticoids in small doses to prevent increased blood clotting. It is advisable to choose medicines Prednisolone, Dexamethasone, Metipred.
2. Immunoglobulin for correcting immunity, weakened by long-term drug therapy.
3. Antiplatelet agents are needed to prevent blood clotting. Such medicines as Curantil, Trental are especially relevant. It will not be superfluous to take aspirin and Heparin.
4. Indirect anticoagulants to control blood viscosity. Doctors recommend the medicine Warfarin.
5. Plasmapheresis provides blood purification in a hospital setting, however, the doses of these medications should be reduced.

With a catastrophic antiphospholipid syndrome, it is necessary to increase the daily dose of glucocorticoids and antiplatelet agents, and it is imperative to purify the blood with an increased concentration of glycoprotein. Pregnancy should proceed under strict medical supervision, otherwise the clinical outcome for a pregnant woman and her child is not the most favorable.

Video: what is AFS

Attention! The information presented in the article is for informational purposes only. The materials of the article do not call for self-treatment. Only a qualified doctor can diagnose and give recommendations for treatment, based on the individual characteristics of a particular patient.

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

What factor is the reason for 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 a wide variety of manifestations, including an increase in blood tone, damage to the skin, the formation of blood clots, etc.

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

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

In the International Classification of Diseases, a separate code for such a syndrome is not allocated, but it belongs to the category of "other coagulation disorders", which is why the ICD-10 code will be D 68.0.

Etiology

The reasons for the development of phospholipid syndromes remain unknown, however, specialists in the field of hematology and rheumatology note the presence of several predisposing factors.

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

• genetic predisposition - the risk of signs of a similar disease is significantly increased when a similar disease is diagnosed in close relatives;
• and other rheumatological pathologies;
• the formation of oncological tumors, regardless of their location and the number of metastases;
• the course of some ailments that affect 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 states;
• uncontrolled intake of certain groups of medicines, in particular interferons, oral contraceptives and psychotropic substances.

Antiphospholipid syndrome is extremely dangerous for pregnant women. 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, however, it is known that in 4% of cases perfectly healthy people are exposed to antiphospholipid syndromes. It is noteworthy that in women, antibodies to phospholipids are detected during laboratory diagnostics several times more often than in men. Moreover, clinicians have established that the older a person is, the more often such a deviation is detected in him, which is why it develops in a child extremely rarely.

Classification

There are several main types of such a disease:

• primary antiphospholipid syndrome - characterized by development in the absence of the course of a particular disease. Its causes remain unclear, but it is believed that it is influenced by aggravated heredity, sluggish infections and drug overdose;
• secondary API - differs in that it occurs due to the course in the human body of any pathological process of an autoimmune, oncological, rheumatic, infectious or drug nature.

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

• catastrophic APS - expressed in a rapid course, the development of failure of all systems and internal organs, which is caused by the formation of blood clots, both large and small;
• APS in combination with vasculitis - in such situations, the course of inflammatory processes in the vessels is observed;
• 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.

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

• seropositive - the main types of antibodies to phospholipids are found in a 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 symptom of the disease is the formation of blood clots, which can be venous (occurring several times more often) and arterial. Veins of the legs, liver, kidneys and retina, as well as cerebral arteries are most often involved in the pathology.

Diagnostics

Due to the fact that the disease has pronounced clinical manifestations, and also has specific laboratory abnormalities, there are no problems with the establishment of the correct diagnosis. Nevertheless, to clarify it, instrumental examinations and a number of manipulations carried out directly by the hematologist are required.

Thus, the primary diagnostic measures include:

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

Laboratory diagnostics include:

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

Instrumental diagnostics of antiphospholipid syndrome is aimed at implementing:

• vascular dopplerography;
• Ultrasound of the fetus;
• ECG and EchoCG;
• cardiography;
• radiography of the peritoneum;
• USDG of arteries and veins of the legs, vessels of the kidneys, liver and head.

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

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

Treatment

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

Drug treatment includes taking:

• direct and indirect anticoagulants;
• glucocorticoids - with catastrophic APS;
• antiplatelet agents;
• antibacterial agents.

In cases of severe course of antiphospholipid syndrome in men, women and children, it is shown:

• intravenous administration of immunoglobulin;
• implementation of plasmapheresis;
• transfusion of fresh frozen plasma.

In addition, treatment should include:

• exercise of moderate physical activity;
• refusal to stay in a motionless state for a long 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, ignorance of clinical signs and inadequate therapy entails the formation of a large number of complications, including:

For pregnant women, pathology is fraught with:

• intrauterine fetal death;
• miscarriages;
• premature birth;
• non-developing pregnancy;
• hemolytic disease of the fetus;
• intrauterine fetal hypoxia.

Prevention and prognosis

Against the background of the fact that the exact causes of the development of the disease remain unknown, preventive clinical recommendations are aimed at adhering to the general rules:

• maintaining a healthy and moderately active lifestyle;
• the use of only those medications that the clinician prescribes;
• 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 - indicated for pregnant women.

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

What is antiphospholipid syndrome?

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

The 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, and secondary. The first variant of antiphospholipid syndrome occurs, as a rule, in persons with a hereditary predisposition. In this case, the presence of the disease in other family members of the patient is characteristic, 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 drugs, in particular, oral contraceptives, quinidine and psychotropic drugs. The 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 anticoagulant systems. As a result of the antiphospholipid syndrome, increased blood coagulability occurs, which is accompanied by the development of various thrombosis.

Signs and symptoms of antiphospholipid syndrome

The 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 by chance during preventive examinations due to Wasserman's false positive 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 (reticular livedo), especially on the legs, feet, thighs, hands, punctate hemorrhages and recurrent venous thrombosis of the lower extremities. With antiphospholipid syndrome, non-healing ulcers can develop on the legs, and if arterial thrombosis occurs, gangrene of the toes.

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

Diagnostics of the 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 screened for this disease.

The first laboratory methods used to diagnose antiphospholipid syndrome are coagulogram (with an estimate of clotting time and activated partial thromboplastin time) and coagulation tests with the addition of various snake venoms. In addition, a decrease in platelets can be detected in a general blood test. 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). Given that these autoantibodies may periodically disappear, a two-time study for their presence is required to diagnose antiphospholipid syndrome with an interval of 6 weeks.

Instrumental diagnostics methods are used to determine the degree of damage to internal organs in antiphospholipid syndrome. For this, ultrasound examination of the abdominal organs, chest x-ray, echocardiography and magnetic resonance imaging of the brain are used. In the case of vein or arterial thrombosis acutely developed as a result of antiphospholipid syndrome, arteriography and ultrasound duplex examination of the vessels of the extremities are useful.

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

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 surgery and obstetricians-gynecologists. A patient with antiphospholipid syndrome can be hospitalized for the selection of antithrombotic therapy, with an increase in blood clotting disorders, and also in the event of a threat of complications of the disease. Women with complicated pregnancy are hospitalized without fail.

If antiphospholipid syndrome is a consequence of other diseases or taking any medications, its cause should be eliminated first. Drug therapy consists in the appointment of anti-thrombotic 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. With antiphospholipid syndrome against the background of other autoimmune manifestations, glucocorticosteroids (prednisolone and methylprednisolone) may be prescribed. Especially often this group of drugs is used to prevent complications of the disease during pregnancy. Methods of extracorporeal detoxification (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 drugs, are possible only after prior consultation with a doctor.

With the development of severe thrombosis against the background of antiphospholipid syndrome, sometimes they resort to surgical interventions consisting in the mechanical removal of a blood clot from the affected vein and artery or in the formation of bypass pathways of blood flow.

Prevention of antiphospholipid syndrome consists, first of all, in the timely examination and treatment of the disease at the slightest suspicion of its presence, preferably before the onset of pregnancy. Long-term use of oral contraceptives is not recommended. All women of reproductive age with relatives who suffer from increased blood clotting and recurrent thrombosis should be screened for antiphospholipid syndrome without fail.

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The embryo becomes a bit human-like, although it still has a tail. The length is 5-13 mm, and the weight is about 0.8 g. The palms, feet and nostrils are formed in the fetus, the rudiments of the tongue and lens of the eyes are laid

1st week of pregnancy - pregnancy calendar

So far, your baby exists only in the form of a "project" (more precisely, half of a "project"), which is represented by one of the many eggs in your ovary. The second half of this same "project" (paternal) has not even had time to take shape in a mature specialist

2nd week of pregnancy - pregnancy calendar

It is too early to talk about pregnancy during this period, as your body is still preparing for it. Now, simultaneously with a change in the hormonal background, a gradual maturation of the next egg is taking place in it.

8th week of pregnancy - pregnancy calendar

The length of the embryo from the crown to the buttocks already reaches 17-20 mm. He has formed auricles, upper lip, nose tip and intestines. The stomach descends into the abdominal cavity. Salivary glands are laid

9th week of pregnancy - pregnancy calendar

The intensity of the uteroplacental blood flow increases and the baby grows intensively. In one week, the embryo turns into a fetus that looks like a little man. The tail disappears. Separated fingers and toes

Antiphospholipid syndrome (APS) is one of the most pressing multidisciplinary problems of modern medicine and is considered as a unique model of autoimmune thrombotic vasculopathy.

The beginning of the study of APS was laid about a hundred years ago in the works of A. Wassermann, devoted to the laboratory method for diagnosing syphilis. During screening studies, it became apparent that a positive Wasserman reaction can be found in many people without clinical signs of syphilitic infection. This phenomenon has received the name "biological false positive Wasserman reaction." It was soon established that the main antigenic component in the Wasserman reaction is a negatively charged phospholipid called cardiolipin. The introduction of the radioimmunological and then the enzyme-linked immunosorbent method (IFM) for the determination of antibodies to cardiolipins (aCL) contributed to a deeper understanding of their role in human diseases. According to modern concepts, antiphospholipid antibodies (aPL) are a heterogeneous population of autoantibodies that interact with negatively charged, less often neutral, phospholipids and / or phospholipid-binding serum proteins. Depending on the method of determination, aPL are conventionally divided into three groups: detected by IFM using cardiolipin, less often other phospholipids; antibodies detectable by functional tests (lupus anticoagulant); antibodies that are not diagnosed using standard methods (antibodies to protein C, S, thrombomodulin, heparan sulfate, endothelium, etc.).

As a consequence of the keen interest in studying the role of aPL and improving methods of laboratory diagnostics, it was concluded that aPL is a serological marker of a peculiar symptom complex, including venous and / or arterial thrombosis, various forms of obstetric pathology, thrombocytopenia, as well as a wide range of neurological, skin, and cardiovascular disorders. ... Beginning in 1986, this symptom complex began to be designated as antiphospholipid syndrome (APS), and in 1994, at the international symposium on APS, the term “Hughes syndrome” was also proposed - after the English rheumatologist who made the greatest contribution to the study of this problem.

The true prevalence of APS in the population is still unknown. Since the synthesis of aPL is possible in normal conditions, low levels of antibodies are often found in the blood of healthy people. According to various sources, the frequency of detection of aCL in the population varies from 0 to 14%, on average it is 2-4%, while high titers are found quite rarely - in about 0.2% of donors. Somewhat more often, aFL is detected in the elderly. At the same time, the clinical significance of aPL in "healthy" individuals (ie, those who do not have obvious symptoms of the disease) is not entirely clear. Often, with repeated analyzes, the level of antibodies elevated in previous determinations is normalized.

An increase in the incidence of aPL was noted in some inflammatory, autoimmune and infectious diseases, malignant neoplasms, while taking medications (oral contraceptives, psychotropic drugs, etc.). There is evidence of an immunogenetic predisposition to increased synthesis of aPL and their more frequent detection in relatives of patients with APS.

It has been proven that aPL is not only a serological marker, but also an important “pathogenetic” mediator that causes the development of the main clinical manifestations of APS. Antiphospholipid antibodies have the ability to affect most of the processes that form the basis for the regulation of hemostasis, the violation of which leads to hypercoagulation. The clinical significance of aPL depends on whether their presence in the blood serum is associated with the development of characteristic symptoms. Thus, APS manifestations are observed only in 30% of patients with positive lupus anticoagulant and in 30-50% of patients with moderate or high levels of aCL. The disease develops mainly at a young age, while APS can be diagnosed in children and even in newborns. Like other autoimmune rheumatic diseases, this symptom complex is more common in women than in men (ratio 5: 1).

Clinical manifestations

The most common and characteristic manifestations of APS are venous and / or arterial thrombosis and obstetric pathology. APS can affect vessels of any caliber and localization - from capillaries to large venous and arterial trunks. Therefore, the spectrum of clinical manifestations is extremely diverse and depends on the localization of thrombosis. According to modern concepts, the basis of APS is a kind of vasculopathy caused by non-inflammatory and / or thrombotic vascular lesions and ending with their occlusion. The APS describes the pathology of the central nervous system, the cardiovascular system, impaired renal function, liver, endocrine organs, and the gastrointestinal tract. Thrombosis of blood vessels of the placenta tend to associate the development of some forms of obstetric pathology ( ).

Venous thrombosis, especially deep vein thrombosis of the lower extremities, is the most typical manifestation of APS, including at the onset of the disease. Blood clots are usually localized in the deep veins of the lower extremities, but can often occur in the hepatic, portal, superficial and other veins. Repeated pulmonary embolism is characteristic, which can lead to the development of pulmonary hypertension. Cases of adrenal insufficiency due to thrombosis of the central vein of the adrenal glands have been described. Arterial thrombosis in general occurs about 2 times less often than venous. They are manifested by ischemia and infarction of the brain, coronary arteries, and peripheral circulation disorders. Intracerebral artery thrombosis is the most frequent localization of arterial thrombosis in APS. Rare manifestations include thrombosis of large arteries, as well as the ascending aorta (with the development of aortic arch syndrome) and the abdominal aorta. A feature of the APS is the high risk of recurrent thrombosis. Moreover, in patients with the first thrombosis in the arterial bed, repeated episodes also develop in the arteries. If the first thrombosis was venous, then repeated thrombosis, as a rule, is noted in the venous bed.

Damage to the nervous system is one of the most severe (potentially fatal) manifestations of APS and includes transient ischemic attacks, ischemic stroke, acute ischemic encephalopathy, episyndrome, migraine, chorea, transverse myelitis, sensorineural hearing loss, and other neurological and psychiatric symptoms. The leading cause of damage to the central nervous system is cerebral ischemia due to thrombosis of cerebral arteries, however, a number of neurological and neuropsychiatric manifestations caused by other mechanisms are distinguished. Transient ischemic attacks (TIA) are accompanied by loss of vision, paresthesia, motor weakness, dizziness, transient general amnesia, and often precede a stroke for many weeks or even months. Recurrence of TIA leads to multi-infarction dementia, which is manifested by cognitive impairment, decreased ability to concentrate and memory, and other symptoms that are nonspecific for APS. Therefore, it is often difficult to differentiate it from senile dementia, metabolic (or toxic) brain damage and Alzheimer's disease. Sometimes cerebral ischemia is associated with thromboembolism, the sources of which are the valves and cavities of the heart or the internal carotid artery. In general, the incidence of ischemic stroke is higher in patients with lesions of the heart valves (especially the left heart).

Headaches are traditionally considered one of the most frequent clinical manifestations of APS. Headaches range from the classic intermittent migraine headaches to persistent, unbearable pain. There are a number of other symptoms (Guillain-Barré syndrome, idiopathic intracranial hypertension, transverse myelitis, parkinsonian hypertonicity), the development of which is also associated with the synthesis of aPL. In patients with APS, veno-occlusive eye diseases are often observed. One of the forms of this pathology is transient loss of vision (amaurosis fugax). Another manifestation - neuropathy of the optic nerve is one of the most common causes of blindness in APS.

Heart disease is represented by a wide range of manifestations, including myocardial infarction, damage to the valvular apparatus of the heart, chronic ischemic cardiomyopathy, intracardiac thrombosis, arterial and pulmonary hypertension. In both adults and children, coronary artery thrombosis is one of the main localizations of arterial occlusion in hyperproduction of aPL. Myocardial infarction occurs in about 5% of aFL-positive patients, and it usually occurs in men younger than 50 years. The most common cardiac sign of APS is damage to the heart valves. It ranges from minimal abnormalities detected only by echocardiography (mild regurgitation, thickening of the valve leaflets) to heart disease (stenosis or insufficiency of the mitral, less often aortic and tricuspid valves). Despite its wide distribution, clinically significant pathology leading to heart failure and requiring surgical treatment is rare (in 5% of patients). However, in some cases, a very severe lesion of the valves with vegetations caused by thrombotic layers, indistinguishable from infective endocarditis, can rapidly develop. Identification of vegetation on the valves, especially if they are combined with hemorrhages in the subungual bed and "drum fingers", creates complex diagnostic problems and the need for a differential diagnosis with infective endocarditis. The APS describes the development of cardiac thrombi that mimic myxoma.

Renal pathology is very diverse. Most patients have only asymptomatic moderate proteinuria (less than 2 g per day), without impaired renal function, but acute renal failure with severe proteinuria (up to nephrotic syndrome), active urinary sediment and arterial hypertension may develop. Kidney damage is mainly associated with intraglomerular microthrombosis and is defined as "renal thrombotic microangiopathy."

Patients with APS have bright and specific skin lesions, primarily reticular livedo (occurring in more than 20% of patients), post-thrombophlebitic ulcers, gangrene of fingers and toes, multiple hemorrhages in the nail bed and other manifestations caused by vascular thrombosis.

With APS, liver damage (Budd-Chiari syndrome, nodular regenerative hyperplasia, portal hypertension), the gastrointestinal tract (gastrointestinal bleeding, spleen infarction, mesenteric vascular thrombosis), and the musculoskeletal system (aseptic bone necrosis) occur.

Among the characteristic manifestations of APS is obstetric pathology, the frequency of which can reach 80%. Fetal loss can occur at any time of pregnancy, but it is more often observed in the II and III trimester. In addition, the synthesis of aPL is associated with other manifestations, including late gestosis, preeclampsia and eclampsia, intrauterine growth retardation, and premature birth. The development of thrombotic complications in newborns from mothers with APS is described, which indicates the possibility of transplacental transmission of antibodies.

Thrombocytopenia is typical for APS. Typically, the platelet count ranges from 70 to 100 x 109 / L and does not require special treatment. The development of hemorrhagic complications is rare and, as a rule, is associated with a concomitant defect in specific blood coagulation factors, renal pathology or an overdose of anticoagulants. Coombs-positive hemolytic anemia is often observed (10%), and Evans syndrome (a combination of thrombocytopenia and hemolytic anemia) is less common.

Diagnostic criteria

The multiple organ symptoms and the need for special confirmatory laboratory tests in some cases make it difficult to diagnose APS. In this regard, in 1999, preliminary classification criteria were proposed, according to which the diagnosis of APS is considered reliable when at least one clinical and one laboratory sign is combined.

Clinical criteria:

• Vascular thrombosis: one or more episodes of thrombosis (arterial, venous, small vessel thrombosis). Thrombosis should be confirmed using instrumental methods or morphologically (morphology - without significant inflammation of the vascular wall).
• Pregnancy pathology can have one of three options:

  One or more cases of intrauterine death of a morphologically normal fetus after 10 weeks of pregnancy;

  One or more episodes of preterm birth of a morphologically normal fetus before 34 weeks of gestation due to severe preeclampsia, or eclampsia, or severe placental insufficiency;

  Three or more consecutive cases of spontaneous abortions before 10 weeks of pregnancy (with the exclusion of anatomical defects of the uterus, hormonal disorders, maternal and paternal chromosomal abnormalities).

Laboratory criteria:

• positive aKL class IgG or IgM in serum in medium and high titers, determined at least twice, with an interval of at least 6 weeks, using a standardized immunoassay method;
• positive lupus anticoagulant detected in plasma at least 6 weeks apart by a standardized method.

Differential diagnosis

Differential diagnosis of APS is carried out with a wide range of diseases occurring with vascular disorders. It should be remembered that with APS, a very large number of clinical manifestations are observed that can mimic various diseases: infective endocarditis, heart tumors, multiple sclerosis, hepatitis, nephritis, etc. APS is sometimes combined with systemic vasculitis. It is believed that APS should be suspected in the development of thrombotic disorders (especially multiple, recurrent, with unusual localization), thrombocytopenia, obstetric pathology in young and middle-aged people in the absence of risk factors for these pathological conditions. It should be excluded in cases of unexplained thrombosis in newborns, in cases of skin necrosis during treatment with indirect anticoagulants and in patients with prolonged activated partial thromboplastin time during screening.

APS was initially described as a variant of systemic lupus erythematosus (SLE). However, it was soon established that APS can also develop in other autoimmune rheumatic and non-rheumatic diseases (secondary APS). Moreover, it turned out that the relationship between hyperproduction of aPL and thrombotic disorders has a more universal character and can be observed in the absence of reliable clinical and serological signs of other diseases. This served as the basis for the introduction of the term "primary API" (PAPS). It is believed that about half of patients with APS suffer from the primary form of the disease. However, it is not completely clear whether PAPS is an independent nosological form. Attention is drawn to the high incidence of PAPS among men (the ratio of men to women is 2: 1), which distinguishes PAPS from other autoimmune rheumatic diseases. Individual clinical manifestations or their combinations are found in patients with PAPS with unequal frequency, which is probably due to the heterogeneity of the syndrome itself. At the moment, three groups of patients with PAPS are conventionally distinguished:

• patients with idiopathic deep vein thrombosis of the lower leg, which is often complicated by thromboembolism, primarily in the pulmonary artery system, leading to the development of pulmonary hypertension;
• young patients (up to 45 years old) with idiopathic strokes, transient ischemic attacks, less often occlusion of other arteries, including coronary arteries; the most striking example of this variant of PAPS is Sneddon's syndrome;
• women with obstetric pathology (repeated spontaneous abortions);

The course of APS, the severity and prevalence of thrombotic complications with it are unpredictable and in most cases do not correlate with changes in the level of APS and disease activity (with secondary APS). In some patients, APS may present with acute, recurrent coagulopathy, often in combination with vasculopathy affecting many vital organs and systems. This served as the basis for the allocation of the so-called "catastrophic APS" (CAFS). To define this condition, the names "acute disseminated coagulopathy-vasculopathy" or "destructive non-inflammatory vasculopathy" were proposed, which also emphasizes the acute, fulminant nature of this variant of APS. The main provoking factor of CAFS is infection. Less often, its development is associated with the abolition of anticoagulants or the intake of certain medications. CAFS occurs in about 1% of patients with APS, but despite ongoing therapy, it ends in death in 50% of cases.

APS treatment

APS prevention and treatment is a complex problem. This is due to the heterogeneity of pathogenetic mechanisms, polymorphism of clinical manifestations, as well as the lack of reliable clinical and laboratory parameters that allow predicting the recurrence of thrombotic disorders. There are no generally accepted international treatment standards, and the recommendations proposed are based mainly on the results of open-label drug trials or retrospective analyzes of disease outcomes.

Treatment with glucocorticoids and cytotoxic drugs for APS is usually ineffective, except in situations where the appropriateness of their appointment is dictated by the activity of the underlying disease (for example, SLE).

The management of patients with APS (as well as with other thrombophilia) is based on the appointment of indirect anticoagulants (warfarin, acenocoumarol) and antiplatelet agents (primarily low doses of acetylsalicylic acid - ASA). This is primarily due to the fact that APS is characterized by a high risk of recurrent thrombosis, which is significantly higher than that in idiopathic venous thrombosis. It is believed that most patients with APS with thrombosis require prophylactic antiplatelet and / or anticoagulant therapy for a long time, and sometimes for life. In addition, the risk of primary and recurrent thrombosis in APS should be reduced by influencing such corrected risk factors as hyperlipidemia (statins: simvastin - simvastol, simlo; lovastatin - rovacor, cardiostatin; pravastatin - lipostat; atorvastatin - avas, liprimar; fibrates: bezafibrate - cholestenorm; fenofibrate - nofibal, grofibrate; ciprofibrate - lipanor), arterial hypertension (ACE inhibitors - kapoten, sinopril, diroton, moex; b-blockers - atenolol, concor, egilok, betalok ZOK, dilatrend, calcium antagonists; normodipine, lacidipine), hyperhomocysteinemia, a sedentary lifestyle, smoking, taking oral contraceptives, etc.

In patients with a high serum aPL level, but without clinical signs of APS (including in pregnant women without a history of obstetric pathology), small doses of ASA (50-100 mg / day) should be limited. The most preferred drugs are aspirin cardio, thrombotic ACC, which have a number of advantages (convenient dosage and the presence of a shell resistant to the action of gastric juice). This form allows not only to provide a reliable antiplatelet effect, but also to reduce the adverse effect on the stomach.

Patients with clinical signs of APS (primarily with thrombosis) require more aggressive anticoagulant therapy. Treatment with vitamin K antagonists (warfarin, phenylin, acenocoumarol) is undoubtedly a more effective, but less safe (compared to ASA) method of preventing venous and arterial thrombosis. The use of vitamin K antagonists requires careful clinical and laboratory monitoring. First, it is associated with an increased risk of bleeding, and the risk of developing this complication, due to its severity, outweighs the benefits of preventing thrombosis. Secondly, in some patients, recurrence of thrombosis is observed after discontinuation of anticoagulant therapy (especially during the first 6 months after discontinuation). Third, in patients with APS, pronounced spontaneous fluctuations in the international normalized ratio (INR) can be observed, which significantly complicates the use of this indicator for monitoring warfarin treatment. However, all of the above should not be an obstacle to active anticoagulant therapy in those patients who need it vital ( ).

The treatment regimen with warfarin consists in prescribing a saturating dose (5-10 mg of the drug per day) for the first two days, and then in selecting the optimal dosage to maintain the target INR. It is advisable to take the entire dose in the morning, before determining the INR. In older people, lower doses of warfarin should be used to achieve the same level of anticoagulation than in younger people. It should be borne in mind that warfarin interacts with a number of drugs, which, when combined, both reduce (barbiturates, estrogens, antacids, antifungal and anti-tuberculosis drugs) and enhance its anticoagulant effect (non-steroidal anti-inflammatory drugs, antibiotics, propranolol, ranitidine, etc. .). Certain dietary recommendations should be made, since foods rich in vitamin K (liver, green tea, leafy vegetables - broccoli, spinach, Brussels sprouts, cabbage, turnips, lettuce) contribute to the development of warfarin resistance. Alcohol is excluded during warfarin therapy.

With insufficient effectiveness of monotherapy with warfarin, it is possible to carry out combined therapy with indirect anticoagulants and low doses of ASA (and / or dipyridamole). Such treatment is most justified in young people without risk factors for bleeding.

In the case of excessive anticoagulation (INR\u003e 4) in the absence of bleeding, it is recommended to temporarily discontinue warfarin until the INR returns to the target level. In the case of hypocoagulation, accompanied by bleeding, it is not enough to prescribe only vitamin K (due to the delayed onset of action - 12-24 hours after administration); fresh frozen plasma or (preferably) prothrombin complex concentrate is recommended.

Aminoquinoline drugs (hydroxychloroquine - plaquenil, chloroquine - delagil) can provide quite effective prevention of thrombosis (at least in the case of secondary APS against the background of SLE). Along with anti-inflammatory action, hydroxychloroquine has certain antithrombotic (inhibits platelet aggregation and adhesion, reduces thrombus size) and hypolipidemic effects.

The central place in the treatment of acute thrombotic complications in APS is occupied by direct anticoagulants - heparin and especially drugs of low molecular weight heparin (fraxiparine, clexane). The tactics of their use do not differ from the generally accepted one.

At CAFS, the entire arsenal of methods of intensive and anti-inflammatory therapy is used, used in critical conditions in patients with rheumatic diseases. The effectiveness of treatment to a certain extent depends on the ability to eliminate the factors that provoke its development (infection, activity of the underlying disease). The appointment of high doses of glucocorticoids in CAFS is not aimed at treating thrombotic disorders, but is determined by the need for therapy of the systemic inflammatory response syndrome (widespread necrosis, adult distress syndrome, adrenal insufficiency, etc.). Usually, pulse therapy is carried out according to the standard scheme (1000 mg of methylprednisolone intravenously per day for 3-5 days), followed by the appointment of glucocorticoids (prednisolone, methylprednisolone) orally (1-2 mg / kg / day). Intravenous immunoglobulin is administered at a dose of 0.4 g / kg for 4-5 days (it is especially effective for thrombocytopenia).

CAFS is the only absolute indication for plasmapheresis sessions, which should be combined with maximum intensive anticoagulant therapy, the use of fresh frozen plasma and pulse therapy with glucocorticoids and cytostatics. Cyclophosphamide (cytoxan, endoxan) (0.5-1 g / day) is indicated for the development of CAFS against the background of exacerbation of SLE and for the prevention of "rebound syndrome" after plasmapheresis sessions. The use of prostacyclin (5 ng / kg / min for 7 days) is justified, however, due to the possibility of the development of "rebound" thrombosis, treatment should be carried out with caution.

The appointment of glucocorticoids to women with obstetric pathology is currently not indicated, due to the lack of data on the benefits of this type of therapy and due to the high frequency of side effects in the mother (Cushing's syndrome, diabetes, arterial hypertension) and the fetus. The use of glucocorticoids is justified only in case of secondary APS against the background of SLE, since it is aimed at treating the underlying disease. The use of indirect anticoagulants during pregnancy is, in principle, contraindicated due to their teratogenic effect.

The standard for the prevention of recurrent fetal loss is small doses of ASA, which are recommended to be taken before, during pregnancy and after the birth of the child (at least for 6 months). During pregnancy, it is desirable to combine small doses of ASA with drugs of low molecular weight heparin. When delivering by cesarean section, the administration of low-molecular-weight heparins is canceled in 2-3 days and resumed in the postpartum period, followed by a transition to indirect anticoagulants. Long-term heparin therapy in pregnant women can lead to the development of osteoporosis, therefore, calcium carbonate (1500 mg) in combination with vitamin D should be recommended to reduce bone loss. It should be borne in mind that treatment with low molecular weight heparin is less likely to cause osteoporosis. One of the limitations to the use of low molecular weight heparins is the risk of developing an epidural hematoma, therefore, if there is a likelihood of premature delivery, treatment with low molecular weight heparins is discontinued no later than 36 weeks of gestation. The use of intravenous immunoglobulin (0.4 g / kg for 5 days every month) has no advantages over standard treatment with ASA and heparin, and is indicated only if standard therapy is ineffective.

Moderate thrombocytopenia in patients with APS does not require special treatment. In secondary APS, thrombocytopenia is well controlled by glucocorticoids, aminoquinoline drugs and, in some cases, by low doses of ASA. The tactics for the treatment of resistant thrombocytopenia, which poses a threat of bleeding, includes the use of high-dose glucocorticoids and intravenous immunoglobulin. If high-dose glucocorticoids fail, splenectomy is the treatment of choice.

In recent years, new antithrombotic agents have been intensively developed, which include heparinoids (heparoid treating, emeran, sulodexide - wessel duet), platelet receptor inhibitors (ticlopidine, tagren, ticlopidin-ratiopharm, clopidogrel, plavix) and other drugs. Preliminary clinical data indicate the undoubted promise of these drugs.

All patients with APS should be under long-term dispensary observation, the primary task of which is to assess the risk of recurrent thrombosis and their prevention. It is necessary to control the activity of the underlying disease (with secondary APS), timely detection and treatment of concomitant pathology, including infectious complications, as well as the impact on the corrected risk factors for thrombosis. Arterial thrombosis, a high incidence of thrombotic complications, and thrombocytopenia were found to be prognostically unfavorable factors in relation to mortality in APS, and the presence of lupus anticoagulant among laboratory markers. The course of APS, the severity and prevalence of thrombotic complications are unpredictable; Unfortunately, universal therapy regimens are lacking. The aforementioned facts, as well as the multiple organ symptoms, require the unification of doctors of various specialties to solve the problems associated with the management of this category of patients.

N. G. Klyukvina, candidate of Medical Sciences, Associate Professor
MMA them. I.M.Sechenova, Moscow