home Calculators Antiphospholipid syndrome is associated with the presence of. Antiphospholipid syndrome - what is it? Diagnosis, tests and clinical recommendations for ATP syndrome. Carrying out urgent treatment

Antiphospholipid syndrome is associated with the presence of. Antiphospholipid syndrome - what is it? Diagnosis, tests and clinical recommendations for ATP syndrome. Carrying out urgent treatment

Disorders of metabolic origin are found in the practice of endocrinologists and not only. These are very different conditions in nature, including those associated with excessive activity of the human body’s own immunity. Dangerous changes occur, and how much depends on the specific diagnosis.

Antiphospholipid syndrome is a pathological process associated with the production of special antibodies to the substance of the same name. Phospholipids are part of the structure of cell membranes, so disruption leads to generalized disorders in the functioning of the body. In particular, the circulatory system suffers.

The “calling card” of the syndrome is a violation of the blood coagulation process, its shift towards hypercoagulation due to the destruction of platelets, precisely those cells that participate in the normal functioning of the body.

In addition, damage to the central nervous system, heart, kidneys and other structures is observed. Huge problems arise during pregnancy, miscarriages and other dangerous complications are possible.

Treatment presents certain difficulties. This is mainly due to the inability to quickly recognize the source of the disorder. However, with competent help, it is possible to correct the disorder and provide a positive outlook.

The pathological process is based on a group of changes. The algorithm for solving the problem is approximately the following:

The human body is influenced by a certain provoking factor. These may include a previous infectious disease, viral or bacterial, there are several options. Systemic disorders of an inflammatory profile, but not of a septic nature.

Also the influence of a toxic factor, poisoning with salts of heavy metals, vapors of other substances. What caused the onset of the pathological process needs to be clarified through diagnostics.

As a result of the influence of a negative factor, a problem like an autoimmune reaction develops. It is false because there is no longer a real threat. Such a failure can be of varying degrees of severity: from minimal, which is practically unnoticeable or has no symptoms at all, to critical violations.
As a result of an autoimmune reaction, a large number of antibodies to phospholipids are produced. These compounds enter the structure of cells and the substances provoke the destruction of membranes, which leads to catastrophic consequences.

It should be noted that in almost 3% of people it is possible to increase the level of antibodies without any health problems. This is not considered a pathology, the body copes with the disorder, but it may well indicate the initial phases of the disorder, so this phenomenon should absolutely not be ignored.

Antibodies to phospholipids cause cell destruction by dissolving the outer membrane. All cytological structures suffer indiscriminately, depending on how immune substances circulate.

As a result of APS syndrome, platelets, the structures of the brain and central nervous system in general, the heart, kidneys are most affected; in pregnant women, the placenta is affected, creating a huge danger to the fetus.

Ultimately, this reaction leads to disruption of the entire body. How dangerous it is depends on the amount of antibodies synthesized and the aggressiveness of the body’s immune response. It is necessary to begin treatment as quickly as possible. Otherwise, it will be impossible to predict complications.

According to statistics, women under the age of 35-45 are mainly affected. They are at increased risk and therefore require careful monitoring, especially during fertile years and during pregnancy.

Classification

The division of the process is carried out according to a complex criterion: etiology plus clinical picture and features of a particular condition.

Accordingly, the following points are determined:

Primary form. Accompanied by spontaneous development. There are no third-party diseases that can cause the disorder. However, it may simply not be detected. Therefore, it makes sense to examine the patient several times at intervals of a couple of months; the diagnosis can be revised.
Secondary pathological process. It occurs much more often. Antiphospholipid syndrome occurs against the background of other diseases. Including atypical ones, such as infectious processes. It is necessary to recognize them in order to then link them with the nature of the deviation.
Idiopathic form. It is determined based on the results of repeated diagnostics. This is a condition whose cause is unclear. At the same time, doctors are forced to deal with the manifestations of the disorder, and not with the disease itself.

As the nature of the disorder becomes clearer, the diagnosis may be revised. The question remains open until further diagnosis. It is considered the least favorable form, since there is no way to influence the root cause.

APL-negative or hidden. Occurs relatively often. Moreover, despite the typical manifestations of the pathological process and the presence of specific antibodies, other markers of the disease are not detected. Lupus anticoagulant and some other substances are not detected, which is strange for such a condition.

It is necessary to conduct a series of laboratory tests to confirm suspicions of a latent form. APS syndrome in a blood test is not the only sign, but the most characteristic, therefore it is assessed in this case.

Critical or catastrophic type. It is characterized by massive hypercoagulation with the development of dangerous complications. A lot of blood clots appear, and without urgent medical care the patient is doomed.

This classification is complex; there are other methods of division, but they are not considered generally accepted, and therefore are used to a much lesser extent.

Symptoms

The clinical picture is extremely heterogeneous, since antiphospholipid antibodies are present throughout the body at once, and changes in a whole group of systems are possible. Signs are named depending on the predominant localization of the disorder.

Circulation

Among the characteristic points:

Thrombosis. Against the background of the disease in question, there is a high probability of the formation of clots that will not allow the tissues and organs to be nourished. This is an extremely dangerous condition, as it is fraught with cell death and gangrene.
Feeling of numbness in the limbs. Manifestation. Occurs as a result of partial blockage. Accompanied by goosebumps and immunity to mechanical stimuli.

Blindness, mono- or binocular. Develops as a result of retinal vein thrombosis. Possible rupture of the vessel, hemophthalmos, but most often simple ischemia begins with destruction of the optic nerve and the formation of a complete and irreversible loss of the ability to see. This is one of the most dangerous scenarios against the background of antiphospholipid syndrome.

Disorders of sensitivity, motor activity. As a result of the same thrombosis.
Changing the shade of fabrics. First to pale, then to violet, crimson or dirty brown. There are several options, depending on which vessels are affected.

Intense pain. When moving and simply trying to lean on the affected leg, use the hand on the side involved in the process. Mainly the limbs are affected.
Necrosis. Tissue death, gangrene.

There are lesions not only of veins, but also of arteries. The development of thrombosis of main and even distant vessels is accompanied by separation of the clot, its migration and ultimately blockage of important trophic pathways (for example) with a rapid fatal outcome. Sometimes the patient does not have time not only to react, but also to understand what happened.

Heart

Cardiac structures are affected just as often. Clinical manifestations of antiphospholipid syndrome include the following symptoms:

Intense pain in the chest. By nature they are either. Which just indicates the ischemic origin of the discomfort. The strength is great, the duration is about several tens of minutes, then the sensation subsides.
Rhythm disturbances. Tachycardia. Acceleration of the beat, as changes progress, heart rate may drop sharply. This is an unfavorable sign.
Dyspnea. Even in a state of complete rest, without any physical activity. With mechanical activity, the manifestation becomes more and more intense.
Nausea.
Dizziness. Central nervous system disorder. Against the background of heart damage, the contractility of the muscular organ decreases; it is not able to sufficiently nourish the brain. This is the cause of poor health. In the future, loss of consciousness is likely, possibly repeated.
Weakness. Asthenic syndrome. Characterized by weakness, drowsiness, and inability to carry out the usual active activities.
A panic attack is possible. A feeling of intense fear with no apparent source of the problem.

The nature of the symptoms resembles complete however, the attack has a high probability of transforming into a heart attack. Patients are required to be hospitalized even before the outcome becomes clear.

Kidneys

Damage to the structures of the excretory tract is accompanied by a group of typical disorders:

Painful sensations. Discomfort appears almost immediately, the intensity is usually insignificant. Character - pulling, aching.
Dysuria. Determined by a lot of options. Violation of the urination process such as retention, a decrease in the amount of daily urine or, on the contrary, an increase in its volume. When excluding diabetes insipidus.

There may be pain during the process of emptying the bladder, and other issues. These are not normal phenomena. However, they are not specific enough; antiphospholipid syndrome is the last thing to be suspected. Hence the importance of carefully collecting anamnesis and analyzing complaints.

Change in urine color. To dark, reddish. This is a clear indication of the development of hematuria or the appearance of protein.
Increase in body temperature. Not always, depending on the specific situation.

Without urgent and high-quality care, kidney failure and other health and life-threatening phenomena are likely.

central nervous system

When the brain is damaged, the following manifestations are observed:

Weakness. Asthenic syndrome.
Impaired coordination of movements. The patient is unable to move normally, control his own activity, or physical manifestations. This is the result of a malfunction of the extrapyramidal system.
Focal neurological symptoms. Depending on which area is affected. There are many forms.
Headache.

Objectively, damage to the central nervous system resembles a stroke or. In different variations.

Skin signs

The main manifestation of APS syndrome from the dermis is the formation of reddish spots of multiple localization of small sizes. The shade varies from pinkish to deep purple or scarlet.

This manifestation is called livedo (reticulated cyanotic pattern), purpura or. This is the result of massive destruction of platelets with the development of hemorrhages.

Signs in pregnant women, reproductive system

Antiphospholipid syndrome poses a tremendous danger to mother and child.

With the development of the pathological process, toxicosis is detected, regardless of the phase of gestation, as well as problems with the liver, brain function, and critical surges in blood pressure (preeclampsia).

As a result, spontaneous termination of pregnancy, death of the child in the womb, and fetal development defects are possible.

The pathological process does not have its own clinical manifestations. It is determined by focal disorders.

Causes

The development of antiphospholipid syndrome is provoked by a group of factors.

Generally speaking, we can name the following categories:

Taking certain medications. Among these are hormonal medications, drugs for treating depression and psychotropic drugs in general.
Diseases of autoimmune origin. From systemic lupus erythematosus to. There are many options. How even rheumatoid arthritis will make itself known, especially without treatment, no one can say.
Infectious processes. From a simple cold with infection of the oropharynx by streptococcus to more serious disorders such as AIDS, tuberculosis and other variants.
Malignant tumors. Not all. Mainly with localization in the organs of the endocrine system, hematopoietic structures, and others.

There is also a hereditary predisposition. It’s difficult to say exactly what role she plays. However, primary forms most often develop as a result of a spontaneous failure, when there is no apparent reason.

Diagnosis of APS

Patients with suspected phospholipid syndrome are examined by hematology specialists. Since the problem is complex, it is possible to involve other doctors in helping. The question remains open and depends on the specific clinical case.

List of events:

Oral questioning of the patient. It is necessary to establish as accurately as possible all deviations in well-being. This is an important point, since symptoms reveal the essence of the pathological process and its localization.

Anamnesis collection. Study of factors that could potentially trigger the onset. Including previous illnesses and other issues.
General blood test.
Coagulogram. With the study of coagulation speed.
Specific tests.
Study of biomaterial for lupus anticoagulant and other substances.

Ultrasound of internal organs, chest x-ray, electroencephalography, ECG, ECHO-CG are indicated as auxiliary techniques.

These are not so much methods for identifying antiphospholipid syndrome, but rather a way to detect its consequences - the degree of disruption of the functioning of internal organs.

Attention:

The APS diagnostic cycle is carried out repeatedly. For at least three weeks, the disease is clarified with parallel restoration of the patient's condition.

Treatment methods

Correction is carried out through medication. Several types of drugs are used.

. Heparin-based products, as well as others.
Actually. In short courses, so as not to provoke the opposite effect. Warfarin and similar.
. Aspirin and more modern options.

Moderate physical activity, cessation of smoking, alcohol, and prolonged sitting in one place are recommended. The problem is also solved through plasmapheresis, if there is a need for it.

The regime plays no less a role. During the first few days, the patient is in the hospital; hospitalization may be extended to a couple of weeks. Further, all activities are carried out on an outpatient basis.

Things are much more complicated during pregnancy. The mother is being preserved and undergoing regular, systematic treatment.

The goal of therapy is not only to relieve symptoms, but also to eliminate the root cause of the abnormal condition. The main forces are directed towards this.

Forecast

With timely treatment, it is positive. The prospects are good. Don't hesitate to get medical help.

Consequences

Among these are necrosis, tissue gangrene, pulmonary embolism, angina pectoris, heart attack, stroke, blindness, renal failure, and disability. Death from complications.

APS or antiphospholipid antibody syndrome (abbreviated as SAFA) is a dangerous condition; without treatment it behaves unpredictably and most likely promises nothing but death.

Therefore, all suspicious symptoms should be grounds for contacting a doctor. Only with timely medical care can you count on recovery.

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 will not suit 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 many laboratory tests, including an analysis for syphilis. And the test turns out to be positive... Then, of course, they will recheck everything and explain that the reaction was false positive and, possibly, due to the presence of antiphospholipid antibodies in the blood serum. However, if this happens, then we can advise you not to panic prematurely, but also not to calm down completely, because antiphospholipid antibodies may someday remind you of themselves.

Video: lectures on APS

general information

About diagnosing APS

APS and other thrombophilias in obstetrics

About antiphospholipid syndrome, what it is, is most often asked by women who have lost a child during pregnancy.

Antiphospholipid syndrome (APS) is a relatively rare pathological condition when the immune system behaves incorrectly, instead of protecting the body, it produces antibodies against its own tissues, namely, so-called “antiphospholipid antibodies” appear in the person’s blood, which are not normally present there should be.

The prevalence of antiphospholipid syndrome is unknown. Antiphospholipid antibodies are found in approximately 5% of healthy people (without signs of any pathology), but the full picture of “autoimmune disease” is detected 5-50 times per 100,000 population. Rarely.

But the disease is insidious:

APS “coexists” with stroke in 14% of cases;
In 11% of cases with myocardial infarction;
in 10% with deep venous thrombosis
And in 9% of cases it is determined in women with frequent miscarriages.

Exactly chronic miscarriage usually makes you think about this rare pathology

So, what do we find in the blood with APS?

Antiphospholipid antibodies are detected in the blood, which should not be present:

lupus anticoagulant;
anticardiolipin antibodies;
anti-beta 2 glycoprotein I

First we need to remember that phospholipids are found in all living tissues of the body and are the main component of cell membranes. Their main function is the transport of fats, cholesterol and fatty acids.

Like most medical terms, the name “antiphospholipid” speaks for itself: some action occurs aimed at destroying phospholipids.

Phospholipids were first noticed in this context at the beginning of the 20th century. It is known that the “Wassermann reaction” is a test for suspected syphilis. During this analysis, a positive test result was observed in some patients (without venereal disease). This is how the term “false-positive Wassermann reaction” appeared in medicine. The question immediately arose: why?

As it turns out, the culprit behind this juicy mess is a phospholipid called cardiolipin. It is a marker antigen for syphilis. That is, in a patient diagnosed with syphilis, the Wassermann test produces antibodies to the phospholipid cardiolipin (anticardiolipin antibodies).
A false-positive reaction to syphilis can be acute (less than 6 months) and chronic (more than 6 months).

But antibodies to phospholipid are produced not only during syphilis. There are a number of conditions in which laboratory results will be similar. For example, rheumatological diseases, some infectious diseases, oncology.

It must be said that antiphospholipid syndrome in rheumatological diseases occupies a “place of honor,” given that in these diseases, in almost 75% of cases, antibodies are produced that are aggressive towards phospholipids. It was even named “Hughes syndrome” in honor of a rheumatologist who was actively involved in this problem.

Today, the variety of antibodies to phospholipids is great, but most often in laboratory studies there are tests for antibodies to cardiolipin, antibodies to lupus coagulant.

What symptoms can there be with antiphospholipid syndrome?

Clinical manifestations of APS are usually increased blood clotting or thrombosis:

thrombotic lesions of the cerebral vascular system;
coronary arteries of the heart;
embolism or thrombosis in the lungs;
blood clots in the arterial or venous system of the legs;
hepatic veins;
renal veins;
ophthalmic arteries or veins;
adrenal glands

A test for antiphospholipids is justified if the patient has a history of deep vein thrombosis (DVT, pulmonary embolism, acute cardiac ischemia or myocardial infarction, stroke (especially when it is repeated) in men<55 лет; женщины <65, а также у женщин с невынашиванием беременностей.

Antiphospholipid syndrome (APS) and pregnancy

During pregnancy, whether APS is primary or secondary plays a role, that is, its presence in the patient was revealed against the background of complete health, or it is a consequence of existing rheumatological diseases.

In any case, both the first and second options need to be treated. Without medical supervision and drug treatment, pregnancy ends sadly in 90% of cases.
But preparation for pregnancy and proper correction during pregnancy always helps patients with APS become a happy mother.

9% of women with recurrent miscarriage suffer from APS.

The thing is that antibodies to phospholipids are attached to the walls of blood vessels. Taking into account the caliber of the vessel, the speed of blood flow, and the state of balance between the coagulation and anticoagulation systems, thrombosis occurs during the formation of placental vessels. This affects the flow of nutrients and oxygen from mother to fetus. Naturally, normal functioning in this condition is impossible.

As a result, there may be complications for both mother and child: feto-placental insufficiency, gestosis, intrauterine fetal death, miscarriage, premature birth, placental abruption.

Antiphospholipid syndrome, diagnosis

Diagnosis is difficult due to the diversity of APS. The caliber of the vessel, its location, where the thrombus occurred, can be different, hence the variability of manifestations.

Thrombosis of the deep veins of the lower extremities is most common, but the veins of the internal organs and superficial veins can also be affected.
Arterial blood clots manifest as cerebral lesions (stroke) and lesions of the coronary vessels of the heart (myocardial infarction). A feature of APS syndrome is the possibility of recurrent thrombosis. Moreover, if the first lesion occurred in the arterial bed, then the second will also be in the artery. Venous thrombosis has the same principle of recurrence.

The clinic (or patient complaints) will indicate the organ where the thrombosis is located. Since blood vessels are everywhere in our body, clinical manifestations can range from headaches to gangrene of the lower extremities.
APS can imitate any disease, so differential diagnosis must be carried out especially carefully.

Laboratory data

thrombocytopenia;
antibodies to cardiolipin (class IgG and IgM) should be determined at least 2 times every 6 weeks;
lupus anticoagulant also with a sampling frequency of 6 weeks.

To make a diagnosis, take into account how many episodes of thrombosis were diagnosed. It is important that research is carried out using instrumental methods.

It is worth contacting a medical institution, if a skin rash appears in the form of small pinpoint manifestations, there is a vascular network (livedo), especially noticeable during cooling, if chronic ulcers of the extremities are annoying, if there are hemorrhages, a history of thrombosis and other symptoms associated with blood vessels.

For pregnant women, a history of previous pregnancy outcomes is very important.
The following outcomes are possible with APS:

intrauterine fetal death;
one or more episodes of labor before 34 weeks as a result of eclampsia, preeclampsia or fetoplacental insufficiency;
more than three spontaneous abortions at a gestational age of up to 10 weeks.

Antiphospholipid syndrome, treatment

Treatment of APS consists of drug prevention of thrombosis. Depending on the severity of the condition, one or more medications are selected.

Usually, regular aspirin is used to prevent thrombosis, but in APS this is not enough, so indirect anticoagulants are used. Most often used. It is a very insidious drug and should never be taken without a doctor’s prescription.

Today, pharmaceutical companies offer a wide range of such drugs. New products entering the pharmaceutical market help protect patients from side effects.

The most dangerous side effect of indirect anticoagulants is bleeding, so the drugs are taken under the constant supervision of a doctor and with the condition of constant visits to the laboratory for blood tests ().

The dose is prescribed gradually. The doctor needs time and dynamic test results to choose the right dose. If we are talking about warfarin, then start with 5 mg and increase.

In acute thrombosis, therapy begins with the injection of direct anticoagulants (heparin). Subsequently, after a day or two, it will be necessary to prescribe an indirect anticoagulant.

The selection of medications is very individual. It is necessary to take into account the severity of the disease, age, presence of concomitant diseases, possible complications, etc.

A separate discussion is the treatment of APS in pregnant women. The difficulty is that indirect anticoagulants are contraindicated. The only medication option is hirudin (antithrombotic drugs that do not cause thrombocytopenia).
The administration of glucocorticoids is also indicated. If immunity is reduced due to hormonal drugs, immunoglobulins are prescribed before the 12th week, during the 24th week and before childbirth.
Taking antiplatelet agents is mandatory.

Of the non-drug methods of treating APS, plasmapheresis will be beneficial.

Constant medical observation and monitoring of laboratory parameters for pregnant women should be regular. This reduces the risk of hematological complications.

An optimistic attitude and the belief that there are no hopeless situations are very important! If antiphospholipid syndrome is treated, then pregnancy can be carried to term! Watch the VIDEO about the motherhood of actress Elena Proklova.

Doctor Vlasenko Natalia Alexandrovna

What tests should I take for APS? Find out in the article!

Antiphospholipid syndrome (APS)– clinical and laboratory symptom complex with recurrent venous and/or arterial thrombosis (thrombophilia state) and obstetric problems associated with overproduction of autoantibodies - antibodies to phospholipids.

According to the literature, among patients with recurrent miscarriage, APS occurs in 27–42% of cases.

An increased level of antibodies to phospholipids affects the processes of implantation, growth, development of the embryo/fetus, the course of pregnancy and the outcome of childbirth.

Antibodies to phospholipids: what tests should I take?

MINIMUM

The diagnosis of antiphospholipid syndrome includes only these tests (so-called criterion antibodies):

antibodies to cardiolipin IgG
antibodies to cardiolipin IgM
antibodies to beta-2-glycoprotein 1 IgG and IgM
lupus anticoagulant (LA 1 screening, LA 1 with normal plasma, LA 2 confirming)

OPTIMUM

However, for a more accurate diagnosis of APS and identification of erased forms, especially with reproductive problems, the determination of minor antibodies is also important. They are less common than the criterion antibodies, but they can also play an independent role in reproductive problems.

Annexin V IgG antibodies
Annexin V IgM antibodies
Antibodies to prothrombin IgG
Antibodies to prothrombin IgM
Determination of the total amount of antibodies to cardiolipin, phosphatidylserine, phosphatidylinositol, phosphatidylic acid and beta2-glycoprotein I - IgG/IgM

MAXIMUM

The third block of tests is autoimmune antibodies to thyroid factors and antibodies to DNA. In case of miscarriage or unsuccessful IVF attempts, it is also important to include them in the examination.

Antibodies to TPO
Antibodies to TG
Antibodies to native DNA
Antibodies to denatured DNA

Antiphospholipid syndrome and diagnosis at the Center

All tests are performed in our specialized CIR laboratory.

We have been diagnosing APS in the field of obstetrics for 20 years, since our collaboration with rheumatologists at the Institute of Rheumatology in Moscow.

To schedule an examination or based on the results, you can contact the doctors of our Center.

Antiphospholipid antibodies: criteria for diagnosing APS

To make a diagnosis of APS, it is necessary to determine elevated levels of autoantibodies twice:

moderate or high levels of IgG and/or IgM antibodies to cardiolipin in the blood serum, detected 2 times within 12 weeks;
moderate or high level of antibodies to beta2-glycoprotein class I IgG and/or IgM in the blood serum, detected 2 times within 12 weeks;
positive test for the presence of lupus anticoagulant in plasma in 2 or more studies obtained at least 6 weeks apart.

(S. Miyakis International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS), 2006).

When diagnosing APS, antibodies of the IgG, IgA and IgM classes are determined, the so-called β-2-GP I-dependent anticardiolipin antibodies, which are detected by ELISA. Cardiolipin in complex with β-2-GP I acts as an antigen.

In APS, antibodies to cardiolipin of the IgG and IgA classes are most common than the IgM class. These antibodies disrupt the formation of the prothrombin-activating complex, which consists of factor X, factor V, phospholipids from platelets and calcium. For the interaction of antiphospholipid antibodies and phospholipids, a serum cofactor is required, which is β-2-glycoprotein I (β-2-GP I). It is currently assumed that antibodies to β-2-GP I play a major role in the pathology of coagulation in patients with APS, while antiphospholipid antibodies themselves are not involved in the pathogenesis of thrombosis in these patients.

β-2-GP I-dependent anticardiolipin antibodies, along with lupus anticoagulant, are the main serological marker of APS and constitute the laboratory criteria for this disease. The titers of these antibodies are usually maximum immediately before the development of thrombosis and decrease slightly immediately after its occurrence, indicating their consumption during the coagulation process. An increase in the titer of β-2-GP I-dependent anticardiolipin antibodies with the development of a clinical picture of thrombosis serves as the basis for the diagnosis of APS.

Antibodies to cardiolipin in low and medium titers are observed in 30-40% of patients with systemic lupus erythematosus (SLE), which allows their use as one of the serological markers of this disease. Their detection does not correlate with age, duration of the disease or special features of its course, including the presence of polyarthritis, serositis or vasculitis. At the same time, the presence of high titers of anticardiolipin antibodies indicates the risk of developing secondary APS against the background of SLE. SLE is not the only rheumatological disease associated with antiphospholipid antibodies. Relatively often, antibodies to cardiolipin are present in patients with vasculitis and vasculitis-like conditions, for example, Behcet's syndrome and Crohn's disease.

At the same time, anticardiolipin antibodies are one of the most common autoantibodies present in clinically healthy individuals. Significant concentrations of anticardiolipin antibodies appear against the background of a wide range of infectious diseases. Among the most common inducers of anticardiolipin antibodies, infections caused by hepatitis C virus, HIV, Epstein-Barr virus, parvovirus B19, streptococci, H. pylori, as well as salmonellosis and urinary tract infections are described. Usually, during infections, low titers of anticardiolipin antibodies of the IgM class appear, which gradually decrease after recovery. This requires repeated determinations of anticardiolipin antibodies with an interval of 1.5 months, which corresponds to the time of renewal of the serum immunoglobulin pool. Oncological diseases and chronic intoxication, including alcoholism, induce antiphospholipid antibodies, which makes it difficult to interpret this indicator.

Antibodies to cardiolipin can be detected in the following diseases: thrombocytopenia, hemolytic anemia, autoimmune diseases, systemic lupus erythematosus, rheumatoid arthritis, rheumatism, periarteritis nodosa, myocardial infarction, stroke, unstable angina, infections (tuberculosis, leprosy, staphylococcal, streptococcal infections, measles , mononucleosis, rubella, AIDS), arterial hypertension, obliterating endarteritis, systemic atherosclerosis, the threat of thrombotic complications, obstetric pathology with the development of APS.

The relative risk of developing strokes, miscarriages, or deep vein thrombosis in patients who have antibodies to cardiolipin in their blood is 2–4 times higher than in patients who do not have them. During pregnancy, due to thromboembolic damage to the trophoblast and placenta, in 95% of cases without treatment, fetal death, miscarriage, placental abruption, fetal malnutrition and hypoxia are observed.

Anticardiolipin antibodies can be detected in both serum and plasma. Inactivation of complement by heat can lead to false-positive results, and repeated freezing leads to a decrease in autoantibody titers.

Normal: IgG – less than 19 IU/ml; IgA – less than 15 IU/ml; IgM – less than 10 IU/ml.
An increase in level characterizes the risk of thrombosis.