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Diagnostic laboratory criteria for antiphospholipid syndrome are: APS syndrome and pregnancy: treatment and diagnosis

Content

Autoimmune diseases are difficult to treat successfully because immune cells fight against 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 must begin with diagnosis. Antiphospholipid syndrome is an autoimmune pathology with persistent resistance of the immune system to phospholipids. Since these are indispensable structures for the formation and strengthening of the skeletal system, improper actions of the immune system can negatively affect the health and functioning of the entire organism. If antiphospholipid antibodies are observed in the blood, the disease does not occur alone; it is accompanied by venous thrombosis, myocardial infarction, stroke, and chronic miscarriage.

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

Antiphospholipid syndrome in men

Extensive medical practice describes cases of illness among representatives of 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 systemic blood flow in individual internal organs and systems is disrupted. Insufficient blood supply can lead to serious health problems such as:

pulmonary embolism;
pulmonary hypertension;
episodes of pulmonary embolism;
thrombosis of the central vein of the adrenal glands;
gradual death of lung, liver tissue, liver parenchyma;
Arterial thrombosis and disorders of the central nervous system cannot be excluded.

Antiphospholipid syndrome in women

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

Antiphospholipid syndrome in pregnant women

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

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

Causes of antiphospholipid syndrome

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

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

Symptoms of antiphospholipid syndrome

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

diagnosis of APS based on the 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 optic neuropathy;
hepatic portal vein thrombosis;
sensorineural hearing loss;
acute coagulopathy;
recurrent hyperkinesis;
dementia syndrome;
transverse myelitis;
thrombosis of cerebral arteries.

Diagnosis of antiphospholipid syndrome

To determine the pathogenesis of the disease, it is necessary to undergo an examination for APS, which requires a blood test for serological markers - lupus anticoagulant and Ab antibodies to cardiolipin. Diagnosis of antiphospholipid syndrome, in addition to testing, includes an anticardiolipin test, aPL, coagulogram, Dopplerometry, 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 indicated. So, pay attention to the following symptom complex:

lupus anticoagulant increases the number of thromboses, while it itself was first diagnosed with systemic lupus erythematosus;
Antibodies to cardiolipin resist natural phospholipids and contribute to their rapid destruction;
antibodies in contact with cardiolipin, cholesterol, phosphatidylcholine are determined by the false-positive Wasserman reaction;
beta2-glycoprotein-1-cofactor-dependent antiphospholipid antibodies become the main cause of symptoms of thrombosis;
antibodies to beta-2-glycoprotein, limiting the patient’s chances of successfully becoming pregnant.
APL-negative subtype without detection of antibodies to phospholipids.

Treatment of antiphospholipid syndrome

If AFLS 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 and includes taking medications from several pharmacological groups. The main goal is to normalize systemic blood circulation, prevent the formation of blood clots with subsequent congestion in the body. So, the main treatment for antiphospholipid syndrome is presented below:

Glucocorticoids in small doses to prevent increased blood clotting. It is advisable to choose medications Prednisolone, Dexamethasone, Metypred.
Immunoglobulin for the correction of immunity weakened by long-term drug therapy.
Antiplatelet agents are necessary to prevent blood clotting. Medicines such as Curantil and Trental are especially relevant. It will not be superfluous to take aspirin and heparin.
Indirect anticoagulants to control blood viscosity. Doctors recommend the drug Warfarin.
Plasmapheresis provides blood purification in a hospital setting, but the doses of these medications should be reduced.

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

Antiphospholipid syndrome - what is it? Diagnosis, tests and clinical recommendations for ATP syndrome

Autoimmune pathology, which is based on the formation of antibodies to phospholipids, which are the main lipid components of cell membranes. Antiphospholipid syndrome can manifest itself as venous and arterial thrombosis, arterial hypertension, valvular heart defects, obstetric pathology (recurrent miscarriage, intrauterine fetal death, preeclampsia), skin lesions, thrombocytopenia, hemolytic anemia. The main diagnostic markers of antiphospholipid syndrome are antibodies to cardiolipin and lupus anticoagulant. Treatment of antiphospholipid syndrome comes down to the prevention of thrombosis, the prescription of anticoagulants and antiplatelet agents.

General information

Antiphospholipid syndrome (APS) is a complex of disorders caused by an autoimmune reaction to phospholipid structures present on cell membranes. The disease was described in detail by the English rheumatologist Hughes in 1986. There are no data on the true prevalence of antiphospholipid syndrome; It is known that insignificant levels of antibodies to phospholipids in blood serum are found in 2-4% of practically healthy individuals, and high titers - in 0.2%. Antiphospholipid syndrome is diagnosed 5 times more often among young women (20-40 years), although men and children (including newborns) can suffer from the disease. As a multidisciplinary problem, antiphospholipid syndrome (APS) attracts the attention of specialists in the field of rheumatology, obstetrics and gynecology, and cardiology.

Reasons

The underlying causes of antiphospholipid syndrome are unknown. Meanwhile, factors predisposing to increased levels of antibodies to phospholipids have been studied and identified. Thus, a transient increase in antiphospholipid antibodies is observed against the background of viral and bacterial infections (hepatitis C, HIV, infectious mononucleosis, malaria, infective endocarditis, etc.). High titers of antibodies to phospholipids are found in patients with systemic lupus erythematosus, rheumatoid arthritis, Sjögren's disease, periarteritis nodosa, and autoimmune thrombocytopenic purpura.

Overproduction of antiphospholipid antibodies can occur with malignant neoplasms, taking medications (psychotropic drugs, hormonal contraceptives, etc.), and discontinuation of anticoagulants. There is information about a genetic predisposition to increased synthesis of antibodies to phospholipids in individuals who carry the HLA DR4, DR7, DRw53 antigens and in relatives of patients with antiphospholipid syndrome. In general, the immunobiological mechanisms of the development of antiphospholipid syndrome require further study and clarification.

Depending on the structure and immunogenicity, “neutral” (phosphatidylcholine, phosphatidylethanolamine) and “negatively charged” (cardiolipin, phosphatidylserine, phosphatidylinositol) phospholipids are distinguished. The class of antiphospholipid antibodies that react with phospholipids includes lupus anticoagulant, antibodies to cardiolipin, beta2-glycoprotein-1-cofactor-dependent antiphospholipids, etc. By interacting with phospholipids of the membranes of vascular endothelial cells, platelets, neutrophils, antibodies cause hemostasis disturbances, expressed in the tendency to hypercoagulation.

Classification

Taking into account the etiopathogenesis and course, the following clinical and laboratory variants of antiphospholipid syndrome are distinguished:

primary– there is no connection with any underlying disease that can induce the formation of antiphospholipid antibodies;
secondary- antiphospholipid syndrome develops against the background of another autoimmune pathology;
catastrophic– acute coagulopathy, occurring with multiple thromboses of internal organs;
AFL-negative a variant of antiphospholipid syndrome, in which serological markers of the disease (Abs to cardiolipin and lupus anticoagulant) are not detected.

Symptoms of antiphospholipid syndrome

According to modern views, antiphospholipid syndrome is an autoimmune thrombotic vasculopathy. In APS, damage can affect vessels of various sizes and locations (capillaries, large venous and arterial trunks), which causes an extremely diverse range of clinical manifestations, including venous and arterial thrombosis, obstetric pathology, neurological, cardiovascular, skin disorders, thrombocytopenia.

The most common and typical sign of antiphospholipid syndrome is recurrent venous thrombosis: thrombosis of the superficial and deep veins of the lower extremities, hepatic veins, portal vein of the liver, retinal veins. Patients with antiphospholipid syndrome may experience recurrent episodes of pulmonary embolism, pulmonary hypertension, superior vena cava syndrome, Budd-Chiari syndrome, and adrenal insufficiency. Venous thrombosis in antiphospholipid syndrome develops 2 times more often than arterial thrombosis. Among the latter, thrombosis of the cerebral arteries predominates, leading to transient ischemic attacks and ischemic stroke. Other neurological disorders may include migraine, hyperkinesis, seizure syndrome, sensorineural hearing loss, ischemic optic neuropathy, transverse myelitis, dementia, and mental disorders.

Damage to the cardiovascular system in antiphospholipid syndrome is accompanied by the development of myocardial infarction, intracardiac thrombosis, ischemic cardiomyopathy, and arterial hypertension. Quite often, damage to the heart valves is observed - from minor regurgitation, detected by echocardiography, to mitral, aortic, tricuspid stenosis or insufficiency. As part of the diagnosis of antiphospholipid syndrome with cardiac manifestations, differential diagnosis with infective endocarditis and cardiac myxoma is required.

Renal manifestations may include both mild proteinuria and acute renal failure. On the part of the gastrointestinal tract, antiphospholipid syndrome causes hepatomegaly, gastrointestinal bleeding, occlusion of mesenteric vessels, portal hypertension, and splenic infarction. Typical lesions of the skin and soft tissues are represented by livedo reticularis, palmar and plantar erythema, trophic ulcers, gangrene of the fingers; musculoskeletal system - aseptic necrosis of bones (femoral head). Hematological signs of antiphospholipid syndrome are thrombocytopenia, hemolytic anemia, and hemorrhagic complications.

In women, APS is often detected in connection with obstetric pathology: repeated spontaneous abortion at various times, intrauterine growth retardation, placental insufficiency, gestosis, chronic fetal hypoxia, premature birth. When managing pregnancy in women with antiphospholipid syndrome, the obstetrician-gynecologist must take into account all possible risks.

Diagnostics

Antiphospholipid syndrome is diagnosed based on clinical (vascular thrombosis, complicated obstetric history) and laboratory data. The main immunological criteria include the detection of medium or high titers of antibodies to cardiolipin of the IgG/IgM class and lupus anticoagulant in the blood plasma twice within six weeks. The diagnosis is considered reliable when at least one main clinical and laboratory criterion is combined. Additional laboratory signs of antiphospholipid syndrome are false-positive RW, positive Coombs test, increased titer of antinuclear factor, rheumatoid factor, cryoglobulins, and antibodies to DNA. A study of CBC, platelets, biochemical blood test, and coagulogram is also indicated.

Pregnant women with antiphospholipid syndrome need monitoring of blood coagulation parameters, dynamic ultrasound of the fetus and

Treatment of antiphospholipid syndrome

The main goal of treatment for antiphospholipid syndrome is to prevent thromboembolic complications. Regular moments include moderate physical activity, avoidance of long periods of immobility, participation in traumatic sports and long air travel. Women with antiphospholipid syndrome should not be prescribed oral contraceptives, and should always consult an obstetrician-gynecologist before planning pregnancy. Pregnant patients are advised to take small doses of glucocorticoids and antiplatelet agents, immunoglobulin administration, and heparin injections under the control of hemostasiogram parameters throughout the entire gestation period.

Drug therapy for antiphospholipid syndrome may include the prescription of indirect anticoagulants (warfarin), direct anticoagulants (heparin, nadroparin calcium, enoxaparin sodium), antiplatelet agents (acetylsalicylic acid, dipyridamole, pentoxifylline). Preventive anticoagulant or antiplatelet therapy for most patients with antiphospholipid syndrome is carried out for a long time, and sometimes for life. In the catastrophic form of antiphospholipid syndrome, the administration of high doses of glucocorticoids and anticoagulants, sessions, transfusion of fresh frozen plasma, etc. is indicated.

Forecast

Timely diagnosis and preventive therapy help avoid the development and recurrence of thrombosis, as well as hope for a favorable outcome of pregnancy and childbirth. In case of secondary antiphospholipid syndrome, it is important to monitor the course of the underlying pathology and prevent infections. Unfavorable prognostic factors are the combination of antiphospholipid syndrome with SLE, thrombocytopenia, a rapid increase in the antibody titer to cardiolipin, and persistent arterial hypertension. All patients diagnosed with antiphospholipid syndrome should be under the supervision of a rheumatologist with periodic monitoring of serological markers of the disease and hemostasiogram parameters.

Antiphospholipid antibody syndrome is an autoimmune disorder in which antibodies are formed in a person's blood to particles of the body's own cells, phospholipids. The pathology increases the risk of thrombosis and in 95% of cases leads to miscarriage.

A complete diagnosis of autoimmune thrombophilia is available only in a specialized laboratory for the pathology of hemostasis at the Medical Women's Center on Taganka. The antiphospholipid syndrome test includes 5 tests and is completed within 24 hours.

Cost of tests for APS syndrome*

Why is a test for APS syndrome prescribed?

Antiphospholipid antibodies attack platelets and vascular lining cells, which provokes thrombosis - blockage of veins and arteries with blood clots. The manifestations of autoimmune thrombophilia are multifaceted - these are heart attacks, strokes, thrombophlebitis, which can develop at a young age, as well as severe complications of pregnancy: miscarriages, gestosis, fetal growth restriction syndrome, placental insufficiency, premature birth.

An analysis for antiphospholipid antibody syndrome should be taken if you are planning a pregnancy, 2 or more cases of intrauterine fetal death, premature birth before 34 weeks, the presence of rheumatic and autoimmune diseases, or a history of arterial or venous thrombosis.

The diagnosis is made on the basis of 1 clinical (cases of thrombosis, obstetric pathologies) and 1 laboratory criterion - high concentration, antibody titer in a blood test.

Specialists

How to get tested for antiphospholipid syndrome

To diagnose autoimmune thrombophilia, venous blood is examined. Before taking blood, it is not recommended to eat food for 4-8 hours in order to obtain reliable results, so the procedure is carried out in the morning:

apply a tourniquet to the patient’s arm;
perform venipuncture;
blood is collected in a test tube and transferred to the Laboratory of Hemostasis Pathology of the MLC for analysis.

Tests for antiphospholipid syndrome - coagulogram, lupus anticoagulant, Ig G immunoglobulins to cardiolipin and other phospholipids, are repeated after 12 weeks.

Repeated analysis shows whether the person really needs treatment - taking anticoagulants, coumarins, or whether the increase in antibody concentration was an immune response to infection, taking certain medications and does not require correction (primary antiphospholipid antibody syndrome).

Video about the problem of APS during pregnancy

Antiphospholipid syndrome is one of the pathologies of hemostasis and the causes of miscarriage, developmental delay or fetal death. Makatsaria A.D. talks about the mechanism of origin and development of APS syndrome and its manifestations, reveals the concepts of “thrombotic storm”, “hypercoagulation”, “hyperhomocysteinemia”, explains when the presence of thrombophilia should be suspected.

obstetrician-gynecologist, hemostasiologist

Decoding indicators

When diagnosing autoimmune thrombophilia, the value of 5 markers is taken into account:

Antibodies to the phospholipid cardiolipin - in APS syndrome, a high titer of Ig A and Ig G is detected, normally 0-12 U/ml.
Antibodies to b2-glycoprotein, a specific blood plasma protein that affects blood clotting processes.
Reference values for all immunoglobulins - G, M and A - are in the range of 0-10 IU/ml.
Lupus anticoagulant (LA) - should not exceed 0.8-1.2 U/ml. Antibodies to cardiolipin and VA are detected simultaneously in 70% of patients with antiphospholipid syndrome.
Antibodies to prothrombin, the 2nd blood clotting factor, are not normally detected.
Annexin-5, a placental anticoagulant protein, is the main cause of placental vascular thrombosis and intrauterine fetal death. Normally absent.

Where to get a blood test for APS syndrome

All specific tests for antiphospholipid antibody syndrome are performed in the express laboratory of the Women's Medical Center on Zemlyanoy Val.

Description:

Antiphospholipid syndrome is a symptom complex based on the development of an autoimmune reaction and the appearance of antibodies to widespread phospholipid determinants present on the membranes of platelets, endothelial cells, and nervous tissue.

Antiphospholipid syndrome was first described in detail in systemic lupus erythematosus by Hughes.

There are several classes of membrane phospholipids, differing in structure and immunogenicity. The most common “neutral” phospholipids in the body are phosphatidylethanolamine (PE) and phosphatidylcholine (PC). “Negatively charged” (anionic) phospholipids - phosphatidylserine (PS), phosphatidylinositol (PI) and cardiolipin (diphosphatidylglycerol) are localized on the inner surface of biomembranes and exposed during cellular activation.

Antibodies to phospholipids are a heterogeneous population of antibodies that react with negatively charged, and less commonly, neutral phospholipids. Antiphospholipid antibodies include the following types of antibodies:

      *lupus anticoagulant is a population of antiphospholipid antibodies of the IgG or IgM class, capable of suppressing phospholipid-dependent coagulation reactions in vitro by interacting with the phospholipid component of the prothrombinase activator complex. Lupus anticoagulant was initially discovered in the blood serum of patients with systemic lupus erythematosus. In systemic lupus erythematosus, the production of lupus anticoagulant is associated, in contrast to the in vitro results, not with bleeding, but with a paradoxical increase in the frequency of thrombosis;
      *antibodies to cardiolipin - an immunologically heterogeneous population of antibodies that react with immobilized negatively charged phospholipid - cardiolipin, which is the main antigen of the Wassermann reaction; antibodies to cardiolipin can belong to various isotypes of immunoglobulins IgG, IgM, IgA;
      *antibodies that react with a mixture of cardiolipin, cholesterol, phosphatidylcholine, determined using an agglutination reaction (false-positive Wasserman reaction);
      *beta2-glycoprotein-1-cofactor-dependent antiphospholipid antibodies (beta2-GP1-cofactor-dependent APL) - suppress the natural anticoagulant activity of beta2-GP1. In antiphospholipid syndrome, the interaction of aPL with phospholipids depends on the cofactor beta2-glycoprotein-1. It has a molecular weight of 50,000,000 Yes and actively binds to phospholipids, DNA, components of platelet and mitochondrial membranes, and heparin. Beta2-GP1 is an important natural anticoagulant that inhibits intrinsic activation of the anticoagulation cascade and platelet aggregation. Suppression of beta2-GP1-cofactor-dependent antibodies is accompanied by the development of thrombosis.

The frequency of detection of antibodies to phospholipids in the blood serum of healthy people ranges from 1 to 12% and may increase in the elderly.

In the serum of healthy people, the level of antibodies to phospholipids is low, while biological membranes are protected from the effects of the latter.

Antiphospholipid syndrome is more common in women than in men.

Symptoms:

Venous and arterial thrombosis.

The most common type is recurrent venous thrombosis. Thrombi are usually localized in the deep veins of the lower extremities, often in the renal and hepatic veins, leading to the development of Budd-Chiari syndrome. Capillaritis is often observed from the deep veins of the lower extremities into the pulmonary artery, which leads to the development of pulmonary hypertension and pulmonary hemorrhages. Thrombosis of the subclavian vein and retinal vein have been described. It is possible to develop superior or inferior vena cava syndrome with thrombosis of the corresponding localization. central vein of the adrenal glands with the subsequent development of hemorrhage and necrosis leads to the development of chronic.
clinically manifested by ischemia and gangrene of the lower extremities, aortic arch syndrome, aseptic necrosis of the femoral head. Thrombosis is caused by the interaction of antiphospholipid antibodies with endothelial cells and a decrease in the antithrombotic activity of the endothelium, suppression of prostacyclin production and increased platelet aggregation, and a decrease in the activity of circulating anticoagulant factors (antithrombin III, protein C and S, beta2-GP1, etc.).

Damage to the central nervous system.

Thrombosis of the cerebral arteries leads to transient ischemic attacks, recurrent strokes, which can manifest not only as paresis and paralysis, but also convulsive syndrome, progressive dementia, and mental disorders. Other neurological disorders are also possible: migraine-like headaches,. It is possible that Sneddon syndrome (a combination of retinal, recurrent thrombosis of cerebral arteries and arterial hypertension) is also a manifestation of antiphospholipid syndrome.

Heart damage.

Cardiac pathology can have a serious prognostic significance and the following clinical manifestations:
- thrombosis of the coronary arteries and the development of myocardial infarction;
- acute or chronic thrombosis of small intramural branches of the coronary arteries, which leads to impaired myocardial contractility;
- damage to the heart valves (mitral valve insufficiency or narrowing of the left atrioventricular orifice, less often - insufficiency of the aortic or tricuspid valves). Some patients may develop thrombotic deposits on the valves (vegetations), which is difficult to differentiate from infectious;
-possible formation of intra-atrial blood clots, which does not resemble the clinical manifestations of the heart;
- labile or stable often develops, which is caused by thrombosis of the renal vessels, renal infarction, thrombosis of the abdominal aorta, intraglomerular renal thrombosis, and the development of fibromuscular dysplasia of the renal arteries.

Kidney damage.

With antiphospholipid syndrome, renal artery thrombosis, renal infarction, as well as intraglomerular microthrombosis (“renal thrombotic microangiopathy”) are often observed with the subsequent development of glomerulosclerosis and.

Liver damage.

Possible thrombosis of the hepatic veins (Budd-Chiari syndrome), arterial damage with the development of liver infarction, and the development of nodular regenerative hyperplasia.

Lung damage.

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 absorbed 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 found 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 also, 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.