Afs syndrome protocol. Antiphospholipid Syndrome - Symptoms and Treatments

Antiphospholipid Syndrome(APS) - a clinical and laboratory symptom complex, including venous and / or arterial thrombosis, various forms of obstetric pathology (primarily recurrent miscarriage), thrombocytopenia, as well as various other neurological, skin, cardiovascular and hematological disorders. A characteristic immunological sign of APS - antibodies to phospholipids - a heterogeneous group of antibodies that react with a wide range of phospholipids and phospholipid-binding proteins. APS most often develops in SLE (secondary APS) or in the absence of another underlying disease (primary APS).

The true prevalence of APS in the population is still not known. The frequency of detection of antibodies to phospholipids in the serum of healthy people varies from 0 to 14%, on average 2-4% (in high titer less than 0.2%). The disease often develops at a young age, can occur in children and even in newborns. In the elderly, the development of APS may be associated with malignant neoplasms. In the general population, APS is more commonly detected in women. However, among patients with primary APS, an increase in the proportion of men is noted.

ETIOLOGY

The causes of APS are not known. An increase in the level (usually transient) of antibodies to phospholipids is observed against the background of a wide range of bacterial and viral infections. However, thrombotic complications in patients with infections develop less frequently than antibodies to phospholipids are detected. There is evidence of an immunogenetic predisposition to hyperproduction of antibodies to phospholipids. An increase in the frequency of detection of antibodies to phospholipids in families of patients with APS was noted; described cases of APS (often primary) in members of the same family.

PATHOGENESIS

Abs against phospholipids bind to phospholipids in the presence of a cofactor, which is β 2 -glycoprotein I, a protein that binds to phospholipids and has anticoagulant activity. Abs to phospholipids present in the serum of APS patients react with antigen formed during the interaction of phospholipid components of the membranes of endothelial and other cells (platelets, neutrophils) and β 2 -glycoprotein I. As a result of this interaction, the synthesis of anticoagulant drugs (prostacyclin, antithrombin III) is suppressed. , annexin V, etc.) and increased formation of procoagulant (thromboxane, tissue factor, platelet activating factor, etc.) mediators, activation of the endothelium (expression of adhesion molecules) and platelets is induced, degranulation of neutrophils occurs.

Abs against phospholipids detected in the serum of patients with infectious diseases usually react with phospholipids in the absence of β 2 -glycoprotein I and do not have the properties described above.

CLASSIFICATION

The following clinical and laboratory variants of APS are distinguished.

Primary APS.

Secondary APS.

. "Catastrophic" AFS.

In some patients, APS is manifested mainly by venous thrombosis, in others by stroke, in others by obstetric pathology or thrombocytopenia. The development of APS does not correlate with the activity of the underlying disease. Approximately half of patients with APS suffer from the primary form of the disease. However, the issue of nosological independence of primary APS is not completely clear. Primary APS can sometimes be an option for the onset of SLE. On the contrary, in some patients with classical SLE at the onset, signs of APS may come to the fore in the future.

Some patients with APS may present with acute recurrent coagulopathy and vasculopathy affecting vital organs and resembling DIC or hemolytic uremic syndrome. This condition has been termed "catastrophic" APS.

CLINICAL PICTURE

Because in based on API non-inflammatory thrombotic lesion of vessels of any caliber and localization lies, the spectrum of clinical manifestations is extremely diverse.

Venous thrombosis is the most common manifestation of APS. Thrombi are usually localized in the deep veins of the lower extremities, but often occur in the hepatic, portal veins, superficial veins, etc. Repeated PE from the deep veins of the lower extremities is characteristic, sometimes leading to pulmonary hypertension. APS (more often primary than secondary) is the second most common cause of Budd-Chiari syndrome. Thrombosis of the central adrenal vein can lead to adrenal insufficiency.

arterial thrombosis. Thrombosis of intracerebral arteries leading to stroke and transient ischemic attacks- the most common localization of arterial thrombosis in APS. Recurrent ischemic strokes, caused by lesions of small-caliber vessels, sometimes proceed without pronounced neurological disorders and can manifest as convulsive syndrome, multi-infarction dementia (resembling Alzheimer's disease), and mental disorders.

Variant APS - Sneddon's syndrome, manifested by recurrent thrombosis of cerebral vessels, livedo reticularis, hypertension and developing in young and middle-aged people. Other neurological disorders include migraine headaches, epileptiform seizures, chorea, and transverse myelitis. Sometimes the neurological deficits in APS resemble those in multiple sclerosis.

Heart valve disease is one of the most common cardiac manifestations of APS. It varies from minimal disturbances detected only by echocardiography (small regurgitation, thickening of the valve leaflets) to severe heart defects (mitral stenosis or insufficiency, less often aortic and tricuspid valves). Some patients quickly develop severe valvular disease with vegetations due to thrombotic overlays, similar to valvular disease in infective endocarditis. Identification of vegetations on the valves, especially if they are combined with hemorrhages in the nail bed and fingers in the form of "drumsticks", dictates the need for differential diagnosis with infective endocarditis. The development of thrombi in the cavity of the heart, mimicking myxoma of the heart, is described. One of possible localizations arterial thrombosis associated with the synthesis of antibodies to phospholipids are the coronary arteries (in men with SLE, this is the most common localization).

Hypertension is a common complication of APS. It can be labile, often associated with livedo reticularis and damage to the cerebral arteries as part of Sneddon's syndrome, or stable, malignant, symptomatic hypertensive encephalopathy. The development of hypertension in APS can be associated with many causes, including renal vascular thrombosis, renal infarction, thrombosis of the abdominal aorta (pseudocoarctation), and intraglomerular thrombosis. A relationship was noted between the hyperproduction of antibodies to phospholipids and the development of fibromuscular dysplasia of the renal arteries. A rare complication of APS is thrombotic pulmonary hypertension, which is associated with both recurrent PE and local ( in situ) pulmonary thrombosis.

Kidney damage in APS is associated with intraglomerular microthrombosis and is referred to as renal thrombotic microangiopathy. Microthrombosis of the glomeruli of the kidneys is considered the cause of the subsequent development of glomerulosclerosis, leading to dysfunction of the organ.

Obstetric pathology is one of the most characteristic signs of APS: recurrent miscarriage, recurrent spontaneous abortions, intrauterine fetal death, preeclampsia. Fetal loss can occur at any stage of pregnancy, but more often in the II and III trimesters.

Skin lesions in APS are characterized by a variety of clinical manifestations(more often livedo reticularis). Less common are skin ulcers, pseudovasculitic lesions (purpura, palmar and plantar erythema, pustules, gangrene of the fingers).

Thrombocytopenia is a typical hematological sign of APS. The development of hemorrhagic complications is rare and is usually associated with a concomitant defect in blood coagulation factors, kidney pathology, or an overdose of anticoagulants. Hemolytic anemia with a positive Coombs reaction is often observed, Evans syndrome (a combination of thrombocytopenia and hemolytic anemia) is less common.

LABORATORY RESEARCH

Laboratory diagnosis of APS is based on the determination of lupus anticoagulant using functional tests and antibodies to cardiolipin using ELISA. In general, lupus anticoagulant has a higher specificity, and anticardiolipin antibodies are more sensitive for diagnosis of APS. Lupus anticoagulant and antibodies to cardiolipin are detected in 30-40% and 40-50% of SLE patients, respectively. In the presence of antibodies to phospholipids, the risk of thrombosis is 40%, while in the absence of antibodies it is not higher than 15%. A method has been developed for determining antibodies that react with β 2 -glycoprotein I, an increase in the level of which correlates better with the development of thrombosis than an increase in the level of antibodies to phospholipids. The course of APS, the severity and prevalence of thrombotic complications in most cases does not depend on the concentration of antibodies to phospholipids.

DIAGNOSTICS

For diagnosis, the provisional classification criteria for APS should be used (Table 46-1).

Table 46-1. Criteria for the diagnosis of APS

. Clinical criteria

. Laboratory criteria

Vascular thrombosis

1 or more episodes of thrombosis of vessels supplying blood to any organ and tissue. With the exception of superficial vein thrombosis, thrombosis must be confirmed by angiography, ultrasonography, or morphology. With morphological confirmation, signs of thrombosis should be observed in the absence of severe inflammatory infiltration of the vascular wall.

Abs to cardiolipin of the IgG or IgM class in medium or high titers, determined at least 2 times within 6 weeks using ELISA, which allows the determination of Abs to β 2 -glycoprotein

obstetric pathology

1 or more unexplained death of a morphologically normal fetus before the 10th month of gestation

1 or more death of a morphologically normal fetus before 34 weeks of gestation due to severe preeclampsia or eclampsia or severe placental insufficiency

3 or more unexplained consecutive spontaneous abortions before the 10th week of gestation, with the exclusion of anatomical and hormonal disorders of the mother's reproductive system or chromosomal disorders in the mother or father

Lupus anticoagulant, detected at least 2 times within 6 weeks by a standardized method, including the following steps

Prolongation of phospholipid-dependent blood coagulation using screening tests (APTT, kaolin test, Russell's viper venom test, prothrombin time, textarine time)

When mixed with normal plasma without platelets, the prolongation of blood clotting time as determined by screening tests is preserved

Normalization of blood clotting time by adding excess phospholipids

Exclusion of other coagulopathies (factor VIII or heparin inhibitors)

A definite diagnosis of APS requires a combination of at least one clinical and one laboratory criterion.

APS should be suspected in cases of thrombotic disorders (especially multiple, recurrent, with unusual localization), thrombocytopenia, obstetric pathology in young and middle-aged people, as well as in unexplained thrombosis in newborns, in case of skin necrosis during treatment with indirect anticoagulants and in patients with a prolonged APTT during a screening study. In APS, a large number of pseudosyndromes are observed that can mimic vasculitis, infective endocarditis, heart tumors, multiple sclerosis, hepatitis, nephritis, etc.

TREATMENT

Prevention and treatment of APS is a complex task (Table 46-2). This is due to the heterogeneity of the pathogenetic mechanisms underlying APS, the polymorphism of clinical manifestations, and the lack of reliable clinical and laboratory parameters to predict the recurrence of thrombotic disorders. The risk of recurrent thrombosis is especially high in young patients with persistently high levels of antibodies to cardiolipin, lupus anticoagulant and with simultaneous detection of antibodies to cardiolipin and lupus anticoagulant, as well as in the presence of recurrent thrombosis and / or obstetric pathology in history, in the presence of other risk factors for thrombotic disorders (hypertension, hyperlipidemia, smoking, taking oral contraceptives), with high SLE activity, with rapid withdrawal of indirect anticoagulants, with a combination of high titers of antibodies to phospholipids with other coagulation disorders.

. Groups patients
Without clinical signs APS, but with a high level of antibodies to phospholipids	No risk factors - low doses acetylsalicylic acid(less than 100 mg/day) ± hydroxychloroquine (100-200 mg/day) (for secondary APS)
	In the presence of risk factors - warfarin (INR less than 2) ± hydroxychloroquine (100-200 mg / day)
With venous thrombosis	Warfarin (INR=2-3) ± hydroxychloroquine (100-200 mg/day)
With arterial thrombosis	Warfarin (INR over 3) ± hydroxychloroquine ± acetylsalicylic acid at low doses (depending on the risk of recurrent thrombosis or bleeding)
with recurrent thrombosis	Warfarin (INR over 3) ± hydroxychloroquine ± low dose acetylsalicylic acid

In addition, there are a number of features in the treatment of APS.

In patients with a high level of antibodies to phospholipids in serum, but without clinical signs of APS (including pregnant women without a history of obstetric pathology), they are limited to prescribing small doses of acetylsalicylic acid (75 mg / day). These persons require careful dynamic monitoring, as the risk of thrombotic complications is very high. Moderate thrombocytopenia usually does not require treatment or is controlled with small doses of GCs.

The management of patients with significant APS is based on the administration of vitamin K antagonists (warfarin) and antiplatelet agents (low doses of acetylsalicylic acid), which are widely used to prevent thrombosis not associated with APS. In patients with both secondary and primary patients with APS, treatment with warfarin, which maintains INR at a level of 2-3 (or more), leads to a significant decrease in the frequency of recurrent thrombotic complications. However, the use of warfarin is associated with a high risk of bleeding. It is advisable to prescribe antimalarial drugs, which, along with anti-inflammatory action, have antithrombotic (suppress platelet aggregation and adhesion, reduce the size of a blood clot) and lipid-lowering activity.

The use of warfarin during pregnancy is contraindicated, as it leads to the development of warfarin embryopathy, characterized by impaired growth of the epiphyses of the bones and hypoplasia of the nasal septum, as well as neurological disorders. Treatment with heparin (especially low molecular weight heparins in standard doses) in combination with low doses of acetylsalicylic acid in women with recurrent miscarriage can increase the rate of successful delivery by about 2-3 times and is significantly superior in effectiveness to glucocorticoid therapy.

Treatment with HA and cytotoxic drugs is not recommended, except in cases of "catastrophic" APS.

FORECAST

The development of APS in SLE reduces the survival of patients. Prognostically unfavorable factors in relation to recurrent thrombosis include thrombocytopenia, arterial thrombosis, and a persistent increase in the level of antibodies to cardiolipin.

This condition is one of the most pressing problems in medicine today, since it affects many organs and systems at the same time, and its diagnosis is difficult in some cases.

In this article, we will try to figure out what kind of symptom complex it is, why it occurs, how it manifests itself, and also consider the principles of diagnosis, treatment and prevention of this condition.

Causes and mechanisms of development of antiphospholipid syndrome

Unfortunately, to date, the reliable causes of this symptom complex are unknown. It is believed that this disease in some cases is genetically determined, this variant is called primary antiphospholipid syndrome, and it is defined as an independent form of the disease. Much more often, antiphospholipid syndrome does not develop on its own, but against the background of any other diseases or pathological conditions, the main of which are:

It can also be the result of a series of medicines: psychotropic medicines, oral hormonal contraceptives, hydralazine, novocainamide and others.

With antiphospholipid syndrome, a large number of autoantibodies to phospholipids are formed in the patient's body, which have several varieties located on the membranes of platelets and endotheliocytes, as well as on nerve cells.

In a healthy person, the frequency of detection of such antibodies is 1-12%, increasing with age. In the diseases mentioned above, the production of antibodies to phospholipids sharply increases, which leads to the development of the antiphospholipid syndrome.

Antibodies to phospholipids have a negative effect on certain structures of the human body, namely:

endotheliocytes (endothelial cells): they reduce the synthesis of prostacyclin in them, which dilates blood vessels and prevents platelet aggregation; inhibit the activity of thrombomodulin, a protein substance that has an antithrombotic effect; inhibit the production of factors that prevent clotting, and initiate the synthesis and release of substances that promote platelet aggregation;
platelets: antibodies interact with these cells, stimulating the formation of substances that enhance platelet aggregation, and also contribute to the rapid destruction of platelets, which causes thrombocytopenia;
humoral components of the blood coagulation system: reduce the concentration in the blood of substances that prevent its coagulation, and also weaken the activity of heparin.

As a result of the effects described above, blood acquires an increased ability to coagulate: blood clots form in the vessels supplying blood to various organs, organs experience hypoxia with the development of appropriate symptoms.

Clinical features of antiphospholipid syndrome

From the side of the skin, the following changes can be determined:

the vascular network on the upper and lower extremities, more often on the hands, is clearly visible during cooling - livedo reticularis;
rash in the form of petechial hemorrhages, resembling outwardly vasculitis;
subcutaneous hematomas;
hemorrhages in the area of the subungual bed (the so-called "symptom of a splinter");
necrosis of skin areas in the area of the distal lower extremities - fingertips;
long-term non-healing ulcerative lesions of the extremities;
redness of the skin of the palms and soles: plantar and palmar erythema;
subcutaneous nodules.

For damage to the vessels of the extremities, the following manifestations are characteristic:

chronic ischemia due to disturbances in blood flow below the site clogged with a thrombus: the limb is cold to the touch, the pulse below the site of thrombosis is sharply weakened, the muscles are atrophied;
gangrene: necrosis of limb tissues as a result of their prolonged ischemia;
thrombosis of deep or superficial veins of the extremities: pain in the extremity, severe swelling, impaired function;
thrombophlebitis: accompanied by severe pain syndrome, fever, chills; along the course of the vein, redness of the skin and painful seals under it are determined.

In the case of localization of a thrombus in large vessels, the following can be determined:

aortic arch syndrome: pressure on the upper limbs is sharply increased, diastolic (“lower”) pressure on the arms and legs varies significantly, noise is determined on the aorta during auscultation;
superior vena cava syndrome: swelling, blue discoloration, dilatation of the saphenous veins of the face, neck, upper torso and upper extremities; may be bleeding from the nose, esophagus, trachea or bronchi;
inferior vena cava syndrome: pronounced, diffuse pain in the lower extremities, groin, buttocks, abdominal cavity; swelling of these parts of the body; dilated saphenous veins.

From the side bone tissue the following changes may occur:

aseptic bone necrosis: necrosis of a portion of bone tissue in the area of the articular surface of the bone; more often observed in the head of the femur; manifested by pain syndrome of indeterminate localization, atrophy of the muscles adjacent to the affected area, impaired movement in the joint;
reversible osteoporosis, not associated with taking glucocorticoids: manifested by pain in the affected area, in the absence of factors that could provoke them.

Manifestations of antifispholipid syndrome on the part of the organ of vision can be:

atrophy of the optic nerve;
hemorrhages in the retina;
thrombosis of arteries, arterioles or retinal veins;
exudation (release of inflammatory fluid) due to blockage of retinal arterioles by a thrombus.

All these conditions are manifested by varying degrees of visual impairment, which is reversible or irreversible.

On the part of the kidneys, the manifestations of antiphospholipid syndrome may be as follows:

kidney infarction: accompanied by a sharp pain in the lower back, decreased diuresis, the presence of blood in the urine; in some cases it is asymptomatic or with minimal clinical manifestations;
thrombosis of the renal artery: suddenly there are sharp pains in the lumbar region, often accompanied by nausea, vomiting, decreased diuresis, stool retention;
renal thrombotic microangiopathy - the formation of microthrombi in the glomeruli - with the subsequent development of chronic renal failure.

With the localization of blood clots in the vessels of the adrenal glands, acute or chronic adrenal insufficiency can develop, as well as hemorrhages and heart attacks in the area of the affected organ can be determined.

Blood clots nervous system It usually manifests itself in the following conditions:

ischemic stroke: accompanied by weakness, dizziness, paresis or paralysis of the skeletal muscles;
migraine: characterized by intense paroxysmal pain in one half of the head, accompanied by nausea and vomiting;
constant excruciating headaches;
psychiatric syndromes.

With the defeat of blood clots of the vessels of the heart are determined:

In case of thrombosis of the liver vessels, its heart attacks, Budd-Chiari syndrome, nodular regenerative hyperplasia are possible.

Very often, with antiphospholipid syndrome, all kinds of obstetric pathology are noted, but it will be discussed below in a separate subsection of the article.

Diagnosis of antiphospholipid syndrome

In 1992, clinical and biological diagnostic criteria for antiphospholipid syndrome were proposed. Clinical criteria include:

habitual miscarriage;
arterial thromboses;
venous thrombosis;
skin lesion - livedo reticularis;
trophic ulcers in the shins;
reduced levels of platelets in the blood;
signs of hemolytic anemia.

The biological criteria are elevated level antibodies to phospholipids - IgG or IgM.

A reliable diagnosis of "antiphospholipid syndrome" is considered if the patient has 2 or more clinical and biological criteria. In other cases, this diagnosis is possible or not confirmed.

V general analysis blood can be detected the following changes:

increased ESR;
reduced platelet level (within * 10 9 / l);
increased content of leukocytes;
sometimes - signs of hemolytic anemia.

V biochemical analysis blood will be found

increased levels of gamma globulin;
in chronic renal failure - elevated levels of urea and creatinine;
in case of liver damage - an increased content of ALT and AST, alkaline phosphatase, bilirubin;
increase in APTT in the analysis of blood coagulability.

Specific immunological blood tests can also be carried out, which determine:

antibodies to cardiolipin, especially IgG in high concentration;
lupus anticoagulant (false-positive or false-negative reactions are not uncommon);
with hemolytic anemia - antibodies to erythrocytes (positive Coombs reaction);
false positive Wasserman reaction;
increased number of T-helpers and B-lymphocytes;
antinuclear factor or antibodies to DNA;
cryoglobulins;
positive rheumatoid factor.

Treatment of antiphospholipid syndrome

In treatment this disease drugs of the following groups can be used:

Antiplatelet agents and anticoagulants of indirect action: aspirin, pentoxifylline, warfarin.
Glucocorticoids (in the case of antiphospholipid syndrome that developed against the background of systemic lupus erythematosus): prednisone; combination with immunosuppressants is possible: Cyclophosphamide, Azathioprine.
Aminoquinoline drugs: Delagil, Plaquenil.
Selective non-steroidal anti-inflammatory drugs: Nimesulide, Meloxicam, Celecoxib.
In obstetric pathology: intravenous immunoglobulin.
B group vitamins.
Preparations of polyunsaturated fatty acids (Omacor).
Antioxidants (Mexicor).

Plasmapheresis is sometimes used in combination with anticoagulant therapy.

To date, they have not received wide application, but the drugs of the following groups are quite promising in the treatment of antiphospholipid syndrome:

monoclonal antibodies to platelets;
anticoagulant peptides;
apoptosis inhibitors;
preparations of systemic enzyme therapy: Wobenzym, Phlogenzym;
cytokines: mainly Interleukin-3.

Indirect anticoagulants (Warfarin) are used to prevent recurrent thrombosis.

In the case of the secondary nature of the antiphospholipid syndrome, it is treated against the background of adequate therapy for the underlying disease.

In 40% of women with repeated cases of intrauterine fetal death, it is the antiphospholipid syndrome that causes them. Blood clots clog the vessels of the placenta, as a result of which the fetus lacks nutrients and oxygen, its development slows down, and in 95% of cases it soon dies. In addition, this disease of the mother can lead to placental abruption or to the development of an extremely dangerous condition, both for the fetus and for the expectant mother - late preeclampsia.

Clinical manifestations of antiphospholipid syndrome during pregnancy are the same as outside this period. Ideally, if this disease was detected in a woman even before pregnancy: in this case, with adequate recommendations from doctors and the diligence of a woman, the probability of having a healthy child is high.

First of all, pregnancy should be planned after the blood counts are normalized as a result of the treatment.

In order to monitor the condition of the placenta and the blood circulation of the fetus, a woman repeatedly undergoes such a study as ultrasound Doppler during pregnancy. In addition, in order to prevent thrombosis in the vessels of the placenta and in general, 3-4 times during pregnancy, she is prescribed a course of drugs that improve metabolic processes: vitamins, microelements, antihypoxants and antioxidants.

If antiphospholipid syndrome is diagnosed after conception, a woman may be given immunoglobulin or heparin in small doses.

Forecast

The prognosis for antiphospholipid syndrome is ambiguous and directly depends both on the timeliness of the start and the adequacy of therapy, and on the discipline of the patient, on his compliance with all doctor's prescriptions.

Which doctor to contact

Antiphospholipid syndrome is treated by a rheumatologist. Since most cases of the disease are associated with the pathology of pregnancy, an obstetrician-gynecologist is involved in the therapy. Since the disease affects many organs, consultation of the relevant specialists is required - a neurologist, nephrologist, ophthalmologist, dermatologist, vascular surgeon, phlebologist, cardiologist.

Lecture by Khasina M. Yu. on the topic "Diagnosis of antiphospholipid syndrome":

Medical Director of the Family Source Center Veronika Ulanova talks about antiphospholipid syndrome:

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Antiphospholipid syndrome - what threatens the disease and how to deal with it?

The composition of all body cells includes esters of higher fatty acids and polyhydric alcohols. These chemical compounds are called phospholipids, they are responsible for maintaining the correct structure of tissues, are involved in metabolic processes and the breakdown of cholesterol. The general state of health depends on the concentration of these substances.

APS syndrome - what is it?

About 35 years ago, rheumatologist Graham Hughes discovered a pathology in which the immune system begins to produce specific antibodies against phospholipids. They attach to platelets and vascular walls, interact with proteins, and enter into metabolic and blood coagulation reactions. Both secondary and primary antiphospholipid antibody syndrome is an autoimmune disease of unknown origin. Young women of reproductive age are more susceptible to this problem.

Antiphospholipid syndrome - causes

Rheumatologists have not yet been able to establish why the disease in question occurs. There is information that antiphospholipid syndrome is more often diagnosed in relatives with a similar disorder. In addition to heredity, experts suggest several other factors that provoke pathology. In such cases, secondary APS develops - the reasons for the production of antibodies are the progression of other diseases that affect the functioning of the immune system. The strategy of therapy depends on the mechanisms of the onset of the disease.

Primary antiphospholipid syndrome

This type of pathology develops independently, and not against the background of some disturbances in the body. Such a syndrome of antiphospholipid antibodies is difficult to treat due to the lack of provoking factors. Often the primary form of the disease is almost asymptomatic and is diagnosed already at the later stages of progression or when complications occur.

Secondary antiphospholipid syndrome

This variant of the autoimmune reaction develops due to the presence of other systemic diseases or certain clinical events. The impetus for the beginning of the pathological production of antibodies can even be conception. Antiphospholipid syndrome in pregnant women occurs in 5% of cases. If the disease in question was diagnosed earlier, gestation will significantly aggravate its course.

Diseases that presumably provoke antiphospholipid syndrome:

viral and bacterial infections;
oncological neoplasms;
nodular periarteritis;
systemic lupus erythematosus.

Antiphospholipid syndrome - symptoms in women

The clinical picture of the pathology is very diverse and non-specific, which makes differential diagnosis difficult. Sometimes the disorder occurs without any signs, but more often the antiphospholipid syndrome manifests itself in the form of recurrent thrombosis of superficial and deep blood vessels (arteries or veins):

Common symptoms in women:

pronounced vascular pattern on the skin (livedo reticularis);
myocardial infarction;
migraine;
suffocation;
pain in the chest;
varicose disease;
thrombophlebitis;
stroke;
arterial hypertension;
acute kidney failure;
ascites;
ischemic attacks;
strong dry cough;
necrosis of bones and soft tissues;
portal hypertension;
gastrointestinal bleeding;
severe liver damage;
spleen infarction;
intrauterine fetal death;
spontaneous miscarriage.

Antiphospholipid syndrome - diagnosis

It is difficult to confirm the presence of the described pathology, because it disguises itself as other diseases and has nonspecific signs. To diagnose a disease, doctors use 2 groups of classification criteria. Examination for antiphospholipid syndrome first involves taking an anamnesis. The first type of evaluation indicators include clinical phenomena:

vascular thrombosis. The medical history should contain one or more cases of damage to the veins or arteries, established instrumentally and laboratory.
obstetric pathology. The criterion is taken into account if there was an intrauterine death of the fetus after the 10th week of gestation or there were premature births before the 34th week of pregnancy in the absence of chromosomal, hormonal and anatomical defects on the part of the parents.

After collecting an anamnesis, the doctor prescribes additional studies. Antiphospholipid syndrome is confirmed when a combination of one clinical symptom and laboratory criterion (minimum) is present. In parallel, a number of measures of differential diagnosis are being carried out. To do this, the specialist recommends undergoing examinations that exclude diseases similar in course.

Antiphospholipid syndrome - analysis

The study of biological fluids helps to identify laboratory signs of the violation presented. The doctor prescribes a blood test for antiphospholipid syndrome to determine the presence of antibodies to cardiolipins and lupus anticoagulant in plasma and serum. Additionally, you can find:

cryoglobulins;
antibodies to erythrocytes;
T- and B-lymphocytes in high concentration;
antinuclear and rheumatoid factor.

How to treat antiphospholipid syndrome?

Therapy for this autoimmune disorder depends on its form (primary, secondary) and the severity of clinical signs. Difficulties arise if an antiphospholipid syndrome is detected in a pregnant woman - treatment should effectively stop the symptoms of the disease, prevent thrombosis, and at the same time not pose a danger to the fetus. To achieve lasting improvements, rheumatologists use a combined therapeutic approach.

Can antiphospholipid syndrome be cured?

It is impossible to completely get rid of the described problem until the causes of its occurrence are established. In antiphospholipid syndrome, it is necessary to use complex treatment aimed at reducing the amount of relevant antibodies in the blood and preventing thromboembolic complications. At severe course disease requires anti-inflammatory therapy.

The main way to eliminate the signs of this pathology is the use of antiplatelet agents and anticoagulants of indirect action:

acetylsalicylic acid (Aspirin and analogues);
Warfarin;
Acenocoumarol;
Phenylin;
Dipyridamole.

How to treat antiphospholipid syndrome - clinical guidelines:

Stop smoking, drinking alcohol and drugs, oral contraceptives.
Adjust the diet in favor of foods rich in vitamin K - green tea, liver, leafy green vegetables.
Fully rest, observe the regime of the day.

If standard therapy is ineffective, the appointment of additional medications is practiced:

aminoquinolines - Plaquenil, Delagil;
direct anticoagulants - Clexane, Fraxiparine;
glucocorticoids - Prednisolone, Methylprednisolone;
platelet receptor inhibitors - Tagren, Clopidogrel;
heparinoids - Emeran, Sulodexide;
cytostatics - Endoxan, Cytoxan;
immunoglobulins (intravenous administration).

Traditional medicine for antiphospholipid syndrome

There are no effective alternative methods of treatment, the only option is to replace acetylsalicylic acid with natural raw materials. Antiphospholipid syndrome cannot be treated with folk recipes because natural anticoagulants are too mild. Before using any alternative means It is important to consult with a rheumatologist. Only a specialist will help alleviate the antiphospholipid syndrome - the doctor's recommendations should be strictly followed.

Tea with the properties of aspirin

dry white willow bark - 1-2 teaspoons;
boiling water - ml.

Rinse the raw materials thoroughly and grind.
Brew willow bark with boiling water, leave for a minute.
Drink the solution as tea 3-4 times a day, you can sweeten to taste.

Antiphospholipid syndrome - prognosis

All patients with a rheumatologist with a diagnosis should be observed for a long time and regularly undergo preventive examinations. How long you can live with antiphospholipid syndrome depends on its form, severity and the presence of concomitant immunological disorders. If primary APS with moderate symptoms is detected, timely therapy and preventive treatment help to avoid complications, the prognosis in such cases is as favorable as possible.

Aggravating factors are the combination of the disease in question with lupus erythematosus, thrombocytopenia, persistent arterial hypertension and other pathologies. In these situations, an antiphospholipid complex syndrome (catastrophic) often develops, which is characterized by an increase in clinical signs and recurrent thrombosis. Some of the consequences can be fatal.

Antiphospholipid syndrome and pregnancy

The described disease is a common cause of miscarriage, so all expectant mothers should undergo a preventive examination and donate blood for a coagulogram. Antiphospholipid syndrome in obstetrics is considered a serious factor provoking fetal death and miscarriage, but its presence is not a sentence. A woman with such a diagnosis is able to bear and give birth to a healthy baby if during pregnancy she follows all the doctor's recommendations and takes antiplatelet agents.

A similar scheme is used when artificial insemination is planned. Antiphospholipid syndrome and IVF are quite compatible, you just have to first take a course of antithrombotic drugs. The use of anticoagulants and antiplatelet agents will continue throughout the gestation period. The effectiveness of such treatment is close to 100%.

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All-Russian public organization

Association of Rheumatologists of Russia

Antiphospholipid syndrome (APS) is a symptom complex that includes recurrent thrombosis (arterial and/or venous), obstetric pathology (more often fetal loss syndrome) and is associated with the synthesis of antiphospholipid antibodies (aPL): anticardiolipin antibodies (aCL) and/or lupus anticoagulant (LA) , and/or antibodies to 2-glycoprotein I (anti-2-GP I). APS is a model of autoimmune thrombosis and belongs to acquired thrombophilias.

ICD code 10 - D68.8 (in the section other blood coagulation disorders; coagulation defects associated with the presence of "lupus anticoagulants"

O00.0 spontaneous in abnormal pregnancy)

Table 1. Diagnostic criteria for APS

One or more clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ. Thrombosis must be confirmed by imaging or Doppler or morphologically, except for superficial venous thrombosis. Morphological confirmation should be presented without the presence of significant inflammation of the vascular wall.

a) one or more cases of intrauterine death of a morphologically normal fetus after 10 weeks of gestation (normal morphological features fetus documented by ultrasound or direct examination of the fetus) or

b) one or more cases premature birth a morphologically normal fetus before 34 weeks' gestation due to severe preeclampsia or eclampsia, or severe placental insufficiency, or

c) three or more consecutive cases of spontaneous abortions before 10 weeks of gestation (exception - anatomical defects of the uterus, hormonal disorders, maternal or paternal chromosomal disorders)

Antibodies to cardiolipin IgG or IgM isotypes, detected in serum in medium or high titers, at least 2 times within 12 weeks, using a standardized enzyme immunoassay.

Antibodies to b2-glycoprotein IIgG and / or IgM isotype detected in serum in medium or high titers at least 2 times within 12 weeks using a standardized enzyme immunoassay.

Plasma lupus anticoagulant, in two or more cases at least 12 weeks apart, determined according to the recommendations of the International Society for Thrombosis and Hemostasis (LA/phospholipid-dependent antibody study group)

a) prolongation of plasma clotting time in phospholipid-dependent coagulation tests: APTT, FAC, prothrombin time, tests with Russell's poisons, textarine time

b) no correction for prolongation of screening test clotting times in mixing tests with donor plasma

c) shortening or correction of the lengthening of the clotting time of screening tests with the addition of phospholipids

e) exclusion of other coagulopathies, such as an inhibitor of coagulation factor VIII or heparin (prolonging phospholipid-dependent blood coagulation tests)

Note. A definite APS is diagnosed by the presence of one clinical and one serological criterion. APS is excluded if aPL without clinical manifestations or clinical manifestations without aPL are detected for less than 12 weeks or more than 5 years. The presence of congenital or acquired risk factors for thrombosis does not rule out APS. Patients should be stratified with a) the presence and b) the absence of risk factors for thrombosis. Depending on aPL positivity, it is recommended to divide APS patients into the following categories: 1. detection of more than one laboratory marker (in any combination); IIa. VA only; II century only akl; only antibodies to b2-glycoprotein I.

A particular aPL profile can be identified as high or low risk for subsequent thrombosis.

Table 2. High and low risk of having different aPL for subsequent thromboses

Lupus anticoagulant (LA) positivity

The positivity of three types of antiphospholipid antibodies (VA + antibodies to cardiolipin (aCL) + anti-β 2-glycoprotein1 antibodies (a-β 2-GP1)

Isolated persistent AKL positivity at high and medium a levels

Isolated intermittent increase in each of the aPL in medium and low levels

a Studied for systemic lupus erythematosus (SLE) only

Recommendations are graded according to the American College of Chest Physicians (ACCP) system: strength of recommendation based on risk/benefit ratio: grade 1: “strong” recommendation = “we recommend”; grade 2 “weak” recommendation = “we advise”. Quality of evidence graded: high quality = A; moderate quality = B; low or very low quality = C, so there are 6 possible grades of recommendation: 1A; 1B; 1C; 2A; 2B; 2C.

Differential Diagnosis APS depends on the existing clinical manifestations. There are a number of genetically determined and acquired diseases that lead to recurrent pregnancy loss, thromboembolic complications, or both (Table 3).

Table 3 Differential Diagnosis antiphospholipid syndrome

SL, distal limb gangrene, skin ulcers, skin necrosis, CNS, kidney damage

Thromboangiitis obliterans (Buerger-Winivarter disease)

Recurrent migratory phlebitis, distal limb gangrene, skin ulcers, skin necrosis, myocardial infarction, mesenteric vascular thrombosis, CNS involvement

Hemorrhagic rashes on the skin, ulcers and necrosis of the skin, kidney damage

Temporal arteritis (Horton's disease)

Retinal artery thrombosis, headaches

Nonspecific aortoarteritis (Takayasu's disease)

Aortic arch syndrome, heart valve disease

TTP (Moszkowitz's disease)

Recurrent thrombosis of vessels of various sizes, thrombocytopenia, hemolytic autoimmune anemia

Recurrent thrombosis of vessels of various sizes, kidney damage, hemolytic anemia, hemorrhages

Ulcers and necrosis of the skin, livedo-vasculitis

Acute rheumatic fever

Formation of heart defects, thrombosis of vessels of various localization (usually CNS and limbs) according to the mechanism of cardiogenic thromboembolism

Thrombosis, hematological disorders, livedo

Livedo, distal limb gangrene, skin ulcers

Hereditary (as a result of mutations in clotting factors, plasma anticoagulants)

Recurrent thrombosis of vessels of various caliber and localization, skin ulcers

Thromboembolic complications, thrombocytopenia, skin ulcers

Tuberculosis, viral hepatitis, etc.

Thromboembolism, transverse myelitis, livedo

The differential diagnosis with thromboembolic disease depends on the vascular bed involved (venous, arterial, or both).

With venous occlusions, if only venous thrombosis or PE is determined, the differential diagnosis includes:

acquired and genetic thrombophilia;

neoplastic and myeloproliferative diseases;

Persons with venous thrombosis younger than 45 years of age with the presence of first-degree relatives with thrombosis at a young age should be investigated for genetic thrombophilia. Today it is clear that the study of aPL should be carried out in some endocrine diseases: Addison's disease and hypopituitarism (Sheehan's syndrome). Although the indication of venous thrombosis is an indicator of thrombophilic status, at the same time, some concomitant clinical manifestations may be a sign of a systemic disease with a higher risk of venous thrombosis. For example, a history of painful mucosal ulcers in the mouth and genitals in young patients with venous thrombosis should suggest a diagnosis of Behçet's disease, which, like APS, affects vessels of any caliber.

If thrombosis is detected only in the arterial bed, the following diseases are excluded:

embolism (with atrial fibrillation, atrial myxoma, endocarditis, cholesterol emboli), myocardial infarction with thrombosis of the ventricles of the heart;

decompression states (Caisson's disease);

Young patients with strokes require special attention, in which more than 18% of cases have aPL in the blood (Kalashnikova L.A.). Some aPL-positive patients may have clinical manifestations similar to multiple sclerosis, which are the result of multiple cerebral infarcts, confirmed by neuroimaging (MRI). A similar type of CNS damage is observed in multiple sclerosis and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. These patients should be carefully questioned about having family members with strokes and dementia at a young age. In the study of autopsies of such cases, multiple deep small cerebral infarcts and diffuse leukoencephalopathy are found. This genetic defect is linked to the 19th chromosome.

With combined thrombosis (arterial and venous), the differential diagnosis includes:

disorders in the fibrinolysis system (dysfibrinogenemia or plasminogen activator deficiency);

myeloproliferative diseases, polycythemia;

paradoxical nocturnal hemoglobinuria;

hyperviscosity of the blood, for example, with Waldström's macroglobulinemia, sickle cell disease, etc.;

When recurrent occlusions of the microvasculature are combined with thrombocytopenia, a differential diagnosis is made between thrombotic microangiopathies (Table 4).

Table 4. Main clinical and laboratory features associated with thrombocytopenia in antiphospholipid syndrome and thrombotic microangiopathies

Antibodies to platelets

Direct Coombs reaction is positive

Note: APS - antiphospholipid syndrome, CAPS - catastrophic APS, TTP - thrombotic thrombocytopenic purpura, DIC - disseminated intravascular coagulation, APTT - activated partial thromboplastin time, PDF - fibrinogen degradation products, ANF - antinuclear factor, aPL - antiphospholipid antibodies.

*negative mixing test (for determining lupus anticoagulant).

# a positive mixing test (when determining lupus anticoagulant).

≠ TTP may be associated with SLE.

§ DIC may be associated with CAPS.

Differential diagnosis between APS and thrombotic angiopathy is often difficult. It must be taken into account that minor thrombocytopenia in APS may be associated with platelet activation and consumption; many clinical and laboratory findings may be common to SLE and TTP. TTP may develop in patients with SLE and, conversely, aPL may occur in TTP, hemolytic uremic syndrome, and HELLP syndrome, and DIC is noted in CAPS. The study of aPL as a screening test is indicated in patients with thrombocytopenia of unknown origin, especially pregnant women with thrombocytopenia, when the risk of hemorrhages due to thrombocytopenia and the risk of thrombosis due to aPL worsen the outcome, both in the fetus and in the mother.

Skin manifestations, among which livedo is the most common, can occur in various rheumatic diseases. Moreover, skin necrosis, skin ulcers, skin discoloration from pallor to redness requires the exclusion of systemic vasculitis, as well as secondary vasculitis on the background of infections. Pyoderma gangrenosum is also often a cutaneous manifestation of systemic rheumatic diseases, but there are case reports.

The pathology of the heart valves requires the exclusion of infective endocarditis, chronic rheumatic fever. Tables 5 and 6 show the signs that occur in these pathologies. As you can see, there are a number of similar features. Rheumatic fever (RF) and APS are two diseases with a similar clinical presentation. The triggering factor in both pathologies is infection. With RL, an infectious agent has been proven - group b-hemolytic streptococcus Streptococcus pyogenes. Molecular mimicry between the microbe and molecules of the heart tissue explains the etiology of LC disease; similar mechanisms also take place in APS. The timing of the development of the disease after infection in LC and APS is different. RL is induced in the first three weeks after infection, there is a clear relationship with a previous streptococcal infection, while in APS most cases develop according to the “hit and run” mechanism, i.e. the development of the disease is delayed in time. The nature of the damage to the heart valves is also different. In APS, valvular stenosis develops rarely and, in contrast to rheumatic stenosis, in these patients, according to our data, there was no adhesion of the commissures, the narrowing of the opening was due to large thromboendocardial overlays and deformation of the valves.

Table 5. Differential diagnosis of valvular heart disease in antiphospholipid syndrome, rheumatic fever, and infective endocarditis

Diffuse thickening or local thickening of the middle part of the valve or its base

Limited valve thickening with superior involvement, chord thickening and fusion, valve calcification

Limited overlays on the atrial or aortic or atrioventricular surface with valve rupture

Table 6. Similar manifestations of antiphospholipid syndrome and acute rheumatic fever(ORL) (Blank M. et al., 2005)

Heart valve deformity

Fibrosis (collagen IV)

CNS damage (chorea)

Streptococcus pyogenes and etc.

Tissue infiltration with lymphocytes

Including T, M protein-reactive cells

Including T reacting with b2 GP1

Expression of adhesion molecules

M-protein and myosin, GlcNA, laminin, b2 GP1

b2 GP1 to cardiolipin and prothrombin, annexin-V, M-protein

Obstetric pathology of APS also requires laboratory confirmation and exclusion of other causes of pregnancy loss. These are genetic thrombophilia, and inflammatory pathology of the genital organs. AFL can be detected when infectious diseases in low or medium positive levels, and to exclude the connection with the infection, repeated studies of aPL after 12 weeks are necessary.

In conclusion, it should be emphasized that APS is an antibody-induced thrombosis, the basis of the diagnosis of which, along with clinical manifestations, is the mandatory presence of serological markers. Obstetric pathology in APS should be considered as a thrombotic complication. A single study of aPL does not allow verification or exclusion of APS.

The management of patients with arterial and/or venous thrombosis and aPL who do not meet the criteria for significant APS (serological markers at low levels) is no different from the management of aPL-negative patients with similar thrombotic outcomes (LE: 1C)

Comments. Data from a systematic review suggest that patients with venous thromboembolism and aPL, even if they do not meet laboratory criteria for the diagnosis of APS, treatment with anticoagulants does not differ from the management of patients with non-aPL thrombosis. Usually, heparins are prescribed first: unfractionated (regular), or low molecular weight, or pentasaccharides, followed by a switch to vitamin K antagonists (VKA) (warfarin).

A vitamin K antagonist (VKA) with an international normalized ratio (INR) target of 2.0-3.0 is recommended for patients with defined APS and first venous thrombosis (LE: 1B)

Despite the fact that clinical guidelines for the diagnosis and treatment of antiphospholipid syndrome have been developed by rheumatologists, it is directly related to obstetrics. Antiphospholipid syndrome during pregnancy leads to recurrent miscarriage, which leads to childlessness of the couple.

Antiphospholipid syndrome, or APS, is a pathology that is characterized by recurrent thrombosis of the venous, arterial, microcirculatory bed, pregnancy pathology with fetal loss and the synthesis of antiphospholipid antibodies (afla): cardiolipin antibodies (aCL) and/or lupus anticoagulant (LA), and/or antibodies to beta2-glycoprotein Ⅰ. APS is a variant of frequently acquired thrombophilia.

ICD 10 revision code - D68.8.

The basis of the pathogenesis of antiphospholipid syndrome is the attack by antibodies of cell membranes. Most often, antiphospholipid syndrome develops in women - 5 times more often than in men.

The manifestation of the syndrome occurs with the occurrence of thrombosis, miscarriage. Often, before the development of gestation, women were unaware of the presence of this pathology and the presence of antibodies in the blood.

Classification

There are several variants of antiphospholipid syndrome. Their main classification is as follows:

Primary - associated with hereditary defects in hemostasis.
Secondary APS arose against the background of autoimmune diseases ( rheumatoid arthritis, systemic lupus erythematosus), vasculitis, organ-specific pathologies ( diabetes, Crohn's disease), oncological processes, drug exposure, infections (HIV, syphilis, malaria), in the final stage of renal failure.
Other API options:

seronegative
catastrophic
other microangiopathic syndromes (DIC, HELLP).

Causes of miscarriage

Pathogenesis of the development of obstetric pathology in APS.

The influence of APS in the development of such pregnancy complications has been proven:

infertility of unknown origin;
early preembryonic losses;
unsuccessful IVF;
miscarriages at different times;
intrauterine fetal death;
postpartum fetal death;
fetal growth retardation syndrome;
preeclampsia and eclampsia;
thrombosis during pregnancy and after childbirth;
fetal malformations.

In the postpartum period, the child also has the consequences of the antiphospholipid syndrome: thrombosis, neurocirculatory disorders with the formation of autism in the future. In 20% of children born to mothers with APS, antiphospholipid antibodies are present in the blood without symptoms, which indicates intrauterine transmission of aPL.

The pathogenic basis for the development of all manifestations of APS during pregnancy is placental decidual vasculopathy, which is caused by a lack of prostaglandin production, placental thrombosis, and a violation of the implantation mechanism. All of these mechanisms prevent pregnancy.

Diagnosis Criteria

There are criteria by which the diagnosis of "Antiphospholipid syndrome" is established. Among the clinical criteria are the following:

Vascular thrombosis of any localization: both venous and arterial, confirmed by visual examination methods. When using histological examination, biopsy specimens should show no signs of inflammation of the vascular wall.
Complications of pregnancy:

one or more episodes of death of a normally developing fetus after 10 weeks of gestation, or
one or more episodes of preterm birth before 34 weeks due to significant preeclampsia, eclampsia, placental insufficiency, or
three or more cases of spontaneous abortions in a row in a period of less than 10 weeks, in the absence of pathologies of the anatomy of the uterus, genetic mutations, genital infections.

The laboratory criteria are:

In the blood, antibodies to cardiolipin, immunoglobulins of classes G and M were detected in medium and high titers, at least 2 times in 12 months.
Antibodies to b2-glycoprotein I classes G and / or M in medium or high titers, at least 2 times a year.
Lupus anticoagulant LA was determined in plasma in 2 more laboratory studies at least 12 months apart. The presence of VA in the blood can be suspected with an increase in APTT in the coagulogram by 2 or more times.

An antibody test is considered highly positive - 60 IU / ml, an average positive response - 20-60 IU / ml, low positive - less than 20 IU / ml.

The diagnosis of Antiphospholipid Syndrome requires the presence of one clinical and one laboratory criterion.

Symptoms

The main symptom of antiphospholipid syndrome is thrombosis. In women, this pathology is manifested by miscarriage. In addition to these obvious signs, women may exhibit additional clinical criteria:

mesh livedo;
a history of migraine, chorea;
trophic ulcerative defects of the lower extremities;
endocarditis, etc.

The catastrophic form of the antiphospholipid syndrome is very difficult. It is accompanied by a clinic of acute renal failure, respiratory distress syndrome, liver failure, impaired cerebral blood flow, thrombosis of large vessels, including pulmonary artery. It is impossible to live with this form for a long time without urgent help.

Treatment

APS is treated by many specialists: rheumatologists, hematologists, obstetricians and gynecologists, cardiologists, cardiac surgeons and others.

The first group of patients

Patients who do not have laboratory-defined signs or clinical symptoms do not need constant laboratory monitoring and continuous anticoagulant therapy. In this group of patients, standard prophylaxis of venous thrombosis is carried out.

Second group

In patients with a high titer of lupus anticoagulant and / or antiphospholipid antibodies of more than 10 IU / ml without thrombosis, specific prophylaxis is required - Aspirin at a dosage of 75-100 mg once a day.

Third group

These people test for antibodies are negative, but there are confirmed cases of thrombosis and a high risk of their formation. These patients are treated with anticoagulants of low molecular weight heparin in therapeutic doses. Immediately after diagnosis is used:

Dalteparin 100 IU/kg twice a day;
Nadroparin 86 IU/kg or 0.1 ml per 10 kg 2 times a day subcutaneously;
Enoxaparin 1 mg/kg 2 times a day subcutaneously;
From the second day, warfarin is prescribed with 5 mg per day.

In patients of this group, heparin therapy is carried out for at least 3 months. At the beginning of therapy, INR is monitored every 4-5 days to maintain the target value of 2.0-3.0.

Fourth group

This group includes people in whom thrombosis occurs against the background of elevated titers of lupus anticoagulant and antiphospholipid antibodies. In this category of patients, Warfarin and a low dose (75-100 mg) of Acetylsalicylic acid are prescribed. Patients at high risk should receive lifelong anticoagulant therapy.

Preconception preparation

Preparation for pregnancy in APS is carried out in 2 consecutive stages. At the first stage, the coagulogram is evaluated, the antigenic components of the blood are determined, and infectious foci are removed and sanitized.

The second stage is the direct preparation for pregnancy and its management. This requires anticoagulant therapy. It is carried out individually for 1-2 menstrual cycles. To do this, you need to classify a woman in one of the following groups:

A seronegative variant of APS with a history of obstetric manifestations of the syndrome. In serum, only antibodies to beta2-glycoprotein I can be detected. In this group, preparation is carried out using the following drugs:

one of the drugs of low molecular weight heparin 1 time / day subcutaneously (dalteparin (Fragmin) 120 antiXa IU / kg or enoxaparin (Clexan) 100 antiXa IU / kg;
fish oil 1-2 capsules 3 times / day;
folic acid 4 mg/day;

If there is no lupus anticoagulant, but APLA is present without thrombosis and obstetric clinical manifestations:

with a moderate APLA titer, Aspirin 75-100 mg / day is prescribed, and with the development of pregnancy, it is canceled and replaced with dipyridomole 50-75 mg / day;
with a high and moderate titer of antiphospholipid antigen, Acetylsalicylic acid 75 mg / day and low molecular weight heparin are combined subcutaneously once a day;
fish oil 1-2 capsules 3 times a day;
folic acid 4 mg/day.

If there is no lupus anticoagulant in the blood, but there is a high or moderate amount of antiphospholipid antigen and there is a clinic of thrombosis and obstetric complications:

one of the LMWHs (Clexane, Fragmin, Fraxiparin) 1 time per day subcutaneously;
Aspirin 75 mg/day with its withdrawal during the development of pregnancy and the appointment of Dipyridamole 50-75 mg/day;
fish oil 1-2 capsules 3 times a day;
folic acid 4 mg/day.

AFLA was found in the woman's plasma and lupus anticoagulant VA was determined from 1.5 to 2 conventional units. Until the normalization of VA, one should refrain from pregnancy. To normalize VA less than 1.2 conventional units, apply:

Clexane 100 antiXa IU/kg or Fragmin 120 antiXa IU/kg once daily subcutaneously;
recommended human immunoglobulin intravenously 25 ml every other day 3 doses, repeat the introduction of the drug at 7-12 weeks of pregnancy, at 24 weeks and the last injection before childbirth;
after the establishment of VA within the normal range, acetylsalicylic acid is prescribed 75 mg / day until pregnancy;
Clexane or Fragmin once a day subcutaneously in the same dosages;
fish oil 1-2 drops. 3 times a day;
folic acid 4 mg/kg.

If VA in the blood is more than 2 conventional units, then conception is delayed for at least 6-12 months. The risk of developing thrombosis in such women is very high. The target value of VA is 1.2 arb. units. Therapy is carried out for at least 6 months.

Laboratory diagnostics and examination when planning pregnancy necessarily include the following indicators of blood coagulation:

platelets - 150-400 * 10 9 / l;
fibrinogen - 2-4 g / l;
INR - 0.7-1.1;
degradation products of fibrinogen and fibrin - less than 5 μg / ml;
d-dimers - less than 0.5 μg / ml;
soluble fibrin monomer complexes should be absent;
protein C - 69.1-134.1%;
antithrombin Ⅲ - 80-120%;
platelet aggregation activity with adenosine diphosphate salt - 50-80%, with adrenaline hydrochloride - 50-80%;
anticardiolipin antibodies - all classes of immunoglobulins less than 10 IU / ml;
VA - negative or less than 0.8-1.2 units;
hyperhomocysteinemia - negative;
mutation FV (Leiden) of the gene responsible for the synthesis of factor V, or mutation G20210A of the gene responsible for the synthesis of factor II - absent;
general urinalysis to determine hematuria;
control over the development of infectious diseases: lymphocytes, ESR.

Management of pregnancy in APS

In order to avoid thrombosis and fetal loss during pregnancy, prevention is necessary - non-drug and drug.

Non-drug:

physical activity stimulates its own tissue plasminogen;
elastic medical jersey 1-2 compression class;
a diet with a lot of vegetable oils, beets, prunes, figs, bananas, since these products have a laxative effect - it is important not to create high blood pressure on the walls of the veins.

Drug prevention of thrombosis during pregnancy

There are several options for prevention, depending on the course of the antiphospholipid syndrome.

There are no serological markers of LA and anticardiolipin antigen, thrombotic complications, antibodies to beta2-glycoprotein I can be determined.

In the first trimester, Clexane or Fragmin is prescribed in a dosage for optimal maintenance of d-dimers and folic acid 4 mg / kg.
Second and third trimesters - Frigmin or Clexane to normal d-dimer numbers, fish oil, Aspirin 75-100 mg / kg with increased platelet aggregation, FFP 10 ml / kg or antithrombin concentrate with a decrease in antithrombin 3 less than 80%.
Before childbirth, Aspirin is canceled 3-5 days in advance, the evening dose of LMWH is changed to FFP 10 mg / kg with heparin 1-2 U for each ml of FFP.
At delivery, the normal level of d-dimers is FFP 10 mg/kg, with a high level before surgery, FFP 5 ml/kg plus heparin 1 U per 1 ml FFP or antithrombin 3 concentrate, during surgery, FFP 5 ml/kg.

With the presence of APLA in the blood and thrombosis or without them, there is no lupus anticoagulant.

1st trimester - Klesan or Fragmin for maintenance normal level d-dimers + folic acid 4 mg / day.
2nd and 3rd trimesters - Clexane or Fragmin in individual dosages + Aspirin 75 mg / day + fish oil 1-2 drops 3 times a day, with a decrease in antithrombin 3 less than 80% of activity - FFP 10 ml / kg or antithrombin concentrate Ⅲ - 10- 50 IU / kg, with an increase in d-dimers more than 0.5 μg / ml - an increase in the dosage of LMWH.
Before childbirth - the abolition of Aspirin for 3-5 days, LMWH is replaced by FFP 10 ml / kg + UFH 1-2 units for each ml of FFP, with an increase in antiphospholipid antibodies, Prednisolone (Methylpred) 1-1.5 mg / kg intravenously is prescribed.
At delivery if normal D-dimers - FFP 10 ml / kg; if d-dimers are elevated, then before surgery FFP 5 ml / kg + UFH 1 unit for each ml of CPG or antithrombin 3 concentrate, during surgery - FFP 5 ml / kg, with a significant increase in antibodies - Prednisolone 1.5-2 ml / kg intravenously.

With an increase in VA from 1.5 to 2 sr.u.

1 trimester - basic intake of Fragmin or Clexane in a dose, as in the previous version + folic acid + human immunoglobulin 25 ml every other day, 3 doses in 7-12 weeks. If there is an increase in VA more than 1.5 units in the first trimester, then the pregnancy should be terminated.
2nd and 3rd trimester - Fragmin and Clexane at a dosage for the normal maintenance of d-dimers + Aspirin 75 mg + fish oil 1-2 drops 3 times a day, with reduced antithrombin - FFP 10 ml / kg or antithrombin concentrate Ⅲ 10-50 IU / kg IV, with an increase in D-dimers - increase the dosage of LMWH, immunoglobulin 25 ml every 1 day 3 times in 24 weeks, if VA is increased from 1.2 to 2 conventional units - Prednisolone 30-60 mg / day IV , from 13 to 34 weeks, a transfer to Warfarin under the control of INR is possible.
Before delivery, if there was Warfarin, then it is canceled 2-3 weeks before, transferred to LMWH, Aspirin is canceled 3-5 days before delivery, FFP 10 ml / kg + UFH 2 units per ml of plasma, Prednisolone - 1.5-2 ml / kg IV, with reduced antithrombin Ⅲ - antithrombin concentrate Ⅲ 10-30 IU / kg.
During childbirth - before surgery FFP 500 ml + UFH 1000 IU, during surgery - FFP 10 ml / kg, Prednisolone 1.5-2 mg / kg IV.

With an increase in VA more than 2 conventional units, pregnancy should be interrupted.

If a woman has developed catastrophic antiphospholipid or HELLP syndrome, then plasmapheresis or plasma filtration may be prescribed.

postpartum period

After delivery, thromboembolism prophylaxis should be resumed after 8-12 hours with Fraxiparine (Nadroparin) - 0.1 ml / 10 kg, Clexane (Enoxaparin) 100 IU / kg, Fragmin (Dalteparin) 120 IU / kg, if there is no bleeding.

If a woman has a history of thrombosis, then therapeutic doses of these drugs are prescribed Fraxiparine - 0.1 ml / 10 kg 2 times a day, Clexane - 100 IU / kg 2 times a day, Fragmin - 120 IU / kg 2 times a day .

The use of LMWH should be continued for at least 10 days. And if there was an episode of proven thromboembolism, then anticoagulants are used for at least 3-6 months.

An increase in the concentration of antigens in the blood requires a consultation with a hematologist or rheumatologist to resolve the issue of hormone therapy.

Price for tests

To identify APS, you can undergo diagnostics on a paid basis. Many private laboratories offer a panel for the determination of antiphospholipid antibodies. In the Invitro laboratory in Moscow, prices at the end of 2018 are as follows:

detection of immunoglobulins G and M to cardiolipin costs 1990 rubles;
diagnosis of secondary APS - price 3170 rubles;
extended serological examination for AFS - 4200 rubles;
laboratory criteria for APS - 3950 rubles.

In the Synevo laboratory in Moscow, the prices for analyzes of this panel vary somewhat:

immunoglobulins G and M to cardiolipin - 960 rubles;
antibodies to beta2-glycoprotein I - 720 rubles;
class G antibodies to phospholipids - 720 rubles;
class M antibodies to phospholipids - 720 rubles.

Approximately the same prices can be offered by other private laboratories in Russian cities.

Antiphospholipid syndrome (synonym: antiphospholipid antibody syndrome; APS) is an autoimmune condition caused by antibodies that are directed against cell membrane phospholipids. The syndrome was first described in 1983 by British rheumatologist Graham Hughes. Antiphospholipid syndrome increases the risk of blood clots (blood clots) in both arteries and veins. In the article we will analyze: APS - what it is, causes and signs.

In some diseases, antibodies are produced in the body that can attack phospholipids - components of cell membranes, which leads to the development of thrombosis

The antiphospholipid syndrome is characterized by the formation of antibodies to the components of one's own cell membranes (phospholipids). Phospholipids are important building blocks of cell membranes in the human body: they are found in platelets, nerve cells, and blood vessel cells. Since many pathogens closely resemble body structures, it can happen that the immune system loses the ability to distinguish between "friends" and "enemies".

Studies show that up to 5% of the human population have antibodies to phospholipids in their blood. Women are much more likely to develop antiphospholipid syndrome than men. The average age of onset of the syndrome ranges from 25 to 45 years.

V international classification diseases of the 10th revision (ICD-10), antiphospholipid antibody syndrome is indicated by the code D68.6.

Causes

The causes of APS are not fully understood. In medicine, there are 2 forms of antiphospholipid syndrome (APS): primary and secondary. The primary form of antiphospholipid syndrome is not caused by a specific organic disease.

Much more common is secondary phospholipid syndrome, which accompanies certain diseases and conditions. At the same time, APS develops due to the fact that pathogens have formations on their surface that are similar to the structures of human cells. As a result, the immune system produces antibodies that bind and eliminate both the pathogen and the body's own lipids. This process is called "molecular mimicry".

The cause of secondary APS can be:

autoimmune diseases (systemic lupus erythematosus, chronic polyarthritis, scleroderma, psoriatic arthritis, etc.);
a number of viral or bacterial infections: HIV, gonorrhea, syphilis, mumps and Lyme disease;
rheumatoid arthritis;
deficiency of vitamin D, vitamin E and cysteine may increase the risk of developing autoimmune diseases;
in rare cases, APS during pregnancy appears due to multiple myeloma or hepatitis;
a very rare cause is long-term use of antiepileptic drugs, quinine and interferon.

Risk factors

People who abuse alcoholic beverages are at risk for the possibility of developing antiphospholipid syndrome.

Main risk factors:

smoking;
overweight;
dehydration;
long-term use of contraceptives (pills);
lack of physical activity;
alcohol abuse;
a diet high in vitamin K-rich foods such as kale, spinach, and cheese;
abuse of arachidonic acid and vegetable omega-6 fatty acids, which are found in edible oils.

Classification

There are four clinical and laboratory forms of APS:

Primary.
Secondary.
Catastrophic (multiple thromboses of internal organs develop in a short time, leading to multiple organ failure).
APL-negative (serological markers of the disease are not determined).

Symptoms

The two main symptoms characteristic of antiphospholipid syndrome are:

arterial and venous thrombosis;
thrombocytopenia.

Venous thrombosis most often occurs in the lower extremities, but can also occur in other parts venous system. Arterial thrombosis occurs predominantly in the vessels of the brain, but can also appear in the arteries of other organs.

Depending on the location of the thrombosis, phospholipid syndrome leads to various complications: pulmonary embolism, heart attacks, kidney infarcts, and strokes. The exact mechanisms of thrombus formation are not fully understood.

Another common symptom, especially in the case of primary antiphospholipid syndrome, is thrombocytopenia - a decrease in the number of platelets, which is characterized by an increased tendency to bleed. Patients may experience paradoxical bleeding in the skin. Women with phospholipid syndrome have an increased risk of early miscarriage.

Visual signs of APL include bluish discoloration of the extremities and skin ulcers that can occur in various parts of the body.

Antiphospholipid syndrome is a common cause of stroke in young patients. If a patient under 45 years of age has a stroke in the absence of risk factors ( arterial hypertension, disorders of lipid metabolism), antiphospholipid syndrome should be excluded.

It is important to understand that not all patients with antiphospholipid antibodies suffer from thrombotic complications. In a large-scale study in which 360 patients with phospholipid antibodies were followed up over a 4-year period, only 9% had venous thrombosis. Other studies have reported a higher incidence of venous and arterial thrombosis.

Diagnostics

The main method for diagnosing antiphospholipid syndrome is the detection of antibodies in the blood plasma.

The symptoms of antiphospholipid syndrome do not allow for a definitive diagnosis, as they may also be associated with other diseases. To detect antiphospholipid syndrome, it is necessary to conduct additional laboratory tests.

In 2006, an expert panel listed the criteria that are still valid and should be used for the definitive diagnosis of antiphospholipid syndrome:

one or more arterial and venous thromboses in a tissue or organ. Blood clots should be confirmed by imaging or histological examination;
one or more unexplained fetal deaths after the 10th week of pregnancy;
several preterm births of morphologically normal newborns at 34 weeks gestation or later;
three or more unexplained spontaneous abortions in a woman before the 10th week of pregnancy.

Laboratory tests and indicators of antiphospholipid syndrome:

increased concentration of anticardiolipin antibodies in the blood in at least two tests at least 12 weeks apart;
a positive test for lupus anticoagulant (in accordance with the recommendations of the international medical community) in blood plasma;
increased concentration of antibodies against beta-2-glycoprotein-1 in two measurements with an interval of 3 months.

In 30-50% of patients, the number of platelets in the blood moderately decreases (70,000-120,000 / μl); only in 5-10% of cases the platelet count is below 50,000/µl. Hemolytic anemia and thrombocytopenic purpura occur in 1% of patients.

A definitive diagnosis of antiphospholipid syndrome can only be made if at least one clinical and laboratory criterion is observed.

Treatment of antiphospholipid syndrome

Aspirin prevents platelets from clumping and counteracts the development of thrombosis and embolism

Due to the lack of large and, accordingly, significant clinical research regarding the causes of the disease, the risk of thrombosis and therapy, there is a lack of clarity regarding the correct treatment strategies, even in expert circles.

The main directions in the therapy of APS are the treatment of acute thrombosis and the prevention of recurrent vascular thrombosis. Patients should be treated promptly as paradoxical bleeding may occur. Late treatment can complicate the course of the disease.

If there are no absolute contraindications, treatment with low dose acetylsalicylic acid is recommended. Aspirin prevents platelets from clumping and thus can counteract the development of thrombosis and embolism. However, there are still no clear results of the study.

Aspirin is supplemented with the introduction of heparin, which prevents blood clotting. For this purpose, Marcumar (indirect anticoagulant) is also used.

Long-term anticoagulant therapy should be used to prevent further thrombosis and embolism. Most effective means- coumarins, which are associated with an increased risk of complications. Lifelong anticoagulation with coumarins is recommended only for patients with phospholipid syndrome and severe thromboembolic complications.

In all patients with antiphospholipid syndrome, it is important to eliminate possible factors that increase the risk of thrombosis: it is recommended to completely stop smoking.

Secondary forms require effective treatment of the underlying disease.

The risk of recurrent thrombosis and occlusion is unfortunately high in patients with confirmed phospholipid syndrome. Therefore, they need to take a long-term (sometimes lifelong) anticoagulant with a vitamin K antagonist.

It is assumed that statins have a moderate antithrombotic effect. Statins are recommended for patients with phospholipid syndrome if they have elevated blood lipids.

Women with antiphospholipid syndrome should refrain from using estrogen-containing drugs that are used to prevent unwanted pregnancy and treat menopausal problems. The use of estrogen significantly increases the risk of blockage of blood vessels.

Treatment of pregnant women with APL

For girls with pregnancy complications, low molecular weight Heparin is administered once a day

Pregnant women are high-risk patients who must be handled with extreme caution. If a woman with antiphospholipid syndrome has not had thrombosis or complications in previous pregnancies, treatment with acetylsalicylic acid is recommended.

Studies show that combined treatment (Aspirin + Heparin) may reduce the risk of further spontaneous abortion. Some international research groups recommend the use of low molecular weight heparin.

Sometimes heparin and low-dose aspirin (100 mg per day) are required. Although heparin has a much shorter duration of action than Marcumar and must be injected under the skin, it is much more effective.

Two to three days after delivery, heparin therapy is resumed and continued for 6 weeks if thromboembolic complications have occurred in the past. If an amniocentesis is performed or C-section, heparin therapy should be interrupted the evening before the procedure.

In addition to heparin therapy, the gynecologist often prescribes progestins to compensate for corpus luteum deficiency. In addition, consistently wearing Grade 2 compression stockings can improve a woman's condition.

For patients with pregnancy complications, low molecular weight heparin is also administered once a day. Low molecular weight heparin, unlike Marcumar, does not cross the placenta and therefore does not affect the fetus.

Complications

Antiphospholipid syndrome is one of the relatively common autoimmune diseases. Complications of APL mainly develop during pregnancy due to the development of placental vascular thrombosis. These complications include:

miscarriages and premature births;
fading of the fetus and its intrauterine death;
premature detachment of the placenta;
anomalies in the development of the fetus;
female infertility;
eclampsia;
gestosis.

If left untreated, pregnancy complications associated with APL occur in 80% of cases.

Smoking is contraindicated for people with antiphospholipid syndrome

Regardless of the form of antiphospholipid syndrome, all patients with this diagnosis should lead a lifestyle that reduces the risk of thromboembolic complications: it is recommended to stop smoking and using other psychotropic drugs.

You need to move more in the fresh air, take enough fluids and do not abuse alcohol. Clinical recommendations largely depend on the patient's condition.

Patients with phospholipid syndrome should refrain from using estrogen-containing contraceptives, as they may contribute to the development of thrombosis.

Pregnancy must be carefully planned due to the increased risk of miscarriage. The treatment of the syndrome must be adjusted during pregnancy to prevent spontaneous abortions and not endanger the fetus. Women who want to become pregnant should be aware of the possible risks and treatment options during pregnancy.

Forecast and prevention

Antiphospholipid syndrome is correlated in elderly people with dementia. The disease also increases the risk of developing kidney disease (renal failure, renal infarction), stroke, myocardial ischemia.

The 10-year mortality rate for patients with APL is 10%, which means that 10% of patients will die as a result of complications of the antiphospholipid antibody syndrome within the next 10 years.

The prognosis is less favorable in women suffering from multiple vascular thrombosis soon after childbirth. There is a danger of multiple narrowing of large and smaller vessels. Massive vasoconstriction can impair blood delivery to vital organs. If the organ fails as a result of narrowing of the lumen of the vessels, the patient may die. The more often a patient experiences thrombosis during his lifetime, the worse the prognosis.

There are no methods for preventing antiphospholipid syndrome. Indirectly, only the development of complications can be prevented. When using anticoagulants, competitive sports should be avoided, soft toothbrushes or an electric razor should be used. The use of new drugs should be reported to the attending physician in advance, as some of them can affect blood clotting.

In case of a stroke heart attack or hemorrhages in the lungs, you need to call an ambulance. The sudden appearance of urine in underwear indicates a kidney infarction, which should also be treated immediately.

Advice! If in doubt, seek the advice of a qualified professional. The earlier treatment begins, the better the prognosis, since with each new thrombosis, the risk of a fatal outcome increases.

A timely visit to a specialist will help prevent complications and, in some cases (secondary antiphospholipid syndrome), completely get rid of the disease.

Content

Autoimmune diseases are difficult to successfully treat, as 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 foreign body trying to exterminate.

What is antiphospholipid syndrome

Any treatment must begin with a diagnosis. Antiphospholipid syndrome is an autoimmune pathology with a stable opposition of immunity to phospholipids. Since these are indispensable structures for the formation and strengthening of the skeletal system, improper actions of the immune system can adversely 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 miscarriage.

This disease may predominate in the primary form, ie. develops independently, as a single ailment of the body. The antiphospholipid syndrome also has a secondary form (HAPS), i.e. becomes a complication chronic disease organism. Alternatively, it can 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 the disease 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 the systemic blood flow is disturbed in certain internal organs and systems. 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 lung, hepatic tissue, liver parenchyma;
arterial thrombosis, disorders of the central nervous system organs are not excluded.

Antiphospholipid syndrome in women

The disease entails catastrophic consequences, so doctors insist on immediate diagnosis, effective treatment. Most clinical pictures 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 the examination for APS show that a huge amount of blood clots is concentrated in the blood. The international code ICD 10 includes the indicated diagnosis, which progresses more often during pregnancy.

Antiphospholipid syndrome in pregnancy

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

If antiphospholipid syndrome develops in pregnant women, for expectant mothers this is fraught with premature and pathological childbirth, miscarriage early term, feto-placental insufficiency, late gestosis, placental abruption, congenital diseases of newborns. APS during pregnancy is a dangerous pathology at any obstetric period, 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 guessing. It has been established that Sneddon's syndrome (it is also called antiphospholipid) may have a genetic predisposition in the presence of the DR7, DRw53, HLA DR4 loci. In addition, the development of the disease against the background of infectious processes of the body is not excluded. Other causes of antiphospholipid syndrome are detailed below:

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

Symptoms of antiphospholipid syndrome

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

diagnosis of APS 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;
thrombosis of the portal vein of the liver;
sensorineural hearing loss;
acute coagulopathy;
recurrent hyperkinesis;
dementia syndrome;
transverse myelitis;
thrombosis of the cerebral arteries.

Diagnosis of 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 Ab antibodies to cardiolipin. Diagnosis of antiphospholipid syndrome, in addition to testing, provides for an anticardiolipin test, APL, 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 itself was first diagnosed with systemic lupus erythematosus;
antibodies to cardiolipin resist natural phospholipids, contribute to 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 main reason 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 VAPS 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, including taking medications from several pharmacological groups. The main goal is to normalize systemic circulation, prevent the formation of blood clots with subsequent congestion of the body. So, the main treatment of antiphospholipid syndrome is presented below:

Glucocorticoids in small doses to prevent increased blood clotting. It is advisable to choose medications Prednisolone, Dexamethasone, Metipred.
Immunoglobulin for the correction of immunity weakened by long-term drug therapy.
Antiplatelet agents are needed to prevent blood clotting. Such medicines as Curantyl, Trental are especially relevant. It will not be superfluous to take aspirin and Heparin.
Indirect anticoagulants to control blood viscosity. Doctors recommend the medical drug Warfarin.
Plasmapheresis provides blood purification in a hospital, however, 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, it is mandatory to cleanse 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.

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