Antiphospholipid syndrome - causes, diagnosis and treatment of the syndrome. APS syndrome and pregnancy: treatment and diagnosis APS syndrome symptoms

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

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

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

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

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

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

Clinical manifestations

The most common and characteristic manifestations of APS are venous and/or arterial thrombosis and obstetric pathology. With APS, vessels of any caliber and localization can be affected - from capillaries to large venous and arterial trunks. Therefore, the spectrum of clinical manifestations is extremely diverse and depends on the localization of thrombosis. According to modern concepts, the basis of APS is a kind of vasculopathy caused by non-inflammatory and / or thrombotic vascular damage and ending in their occlusion. Within the framework of APS, the pathology of the central nervous system, cardiovascular systems s, impaired function of the kidneys, liver, endocrine organs, gastrointestinal tract. Placental thrombosis tends to be associated with the development of certain forms of obstetric pathology ( table 1).

Venous thrombosis, especially deep vein thrombosis lower extremities, -most typical manifestation APS, including at the onset of the disease. Thrombi are usually localized in the deep veins of the lower extremities, but can often occur in the hepatic, portal, superficial and other veins. Repeated pulmonary embolisms are characteristic, which can lead to the development of pulmonary hypertension. Cases of development of adrenal insufficiency due to thrombosis of the central vein of the adrenal glands are described. In general, arterial thromboses occur about 2 times less often than venous ones. They are manifested by ischemia and cerebral infarction, coronary arteries, peripheral circulatory disorders. Thrombosis of the intracerebral arteries is the most common localization of arterial thrombosis in APS. Rare manifestations include thrombosis of the large arteries, as well as the ascending aorta (with the development of aortic arch syndrome) and the abdominal aorta. A feature of APS is a high risk of thrombosis recurrence. At the same time, in patients with the first thrombosis of the arterial bed, repeated episodes also develop in the arteries. If the first thrombosis was venous, then repeated thromboses, as a rule, are noted in the venous bed.

Defeat nervous system refers to the most severe (potentially fatal) manifestations of APS and includes transient ischemic attacks, ischemic stroke, acute ischemic encephalopathy, episyndrome, migraine, chorea, transverse myelitis, sensorineural hearing loss and other neurological and psychiatric symptoms. The leading cause of CNS damage is cerebral ischemia due to cerebral artery thrombosis, however, a number of neurological and neuropsychic manifestations due to other mechanisms are distinguished. Transient ischemic attacks (TIA) are accompanied by loss of vision, paresthesias, motor weakness, dizziness, transient general amnesia, and often many weeks and even months precede a stroke. Recurrence of TIA leads to multi-infarct dementia, which is manifested by cognitive impairment, decreased ability to concentrate and memory, and other symptoms that are not specific to APS. Therefore, it is often difficult to differentiate it from senile dementia, metabolic (or toxic) brain damage, and Alzheimer's disease. Sometimes cerebral ischemia is associated with thromboembolism, the sources of which are the valves and cavities of the heart or the internal carotid artery. In general, the frequency of ischemic stroke is higher in patients with valvular heart disease (especially the left side).

Headaches are traditionally regarded as one of the most common clinical manifestations AFS. The nature of headaches varies from classic intermittent migraine headaches to constant, unbearable pain. There are a number of other symptoms (Guillain-Barré syndrome, idiopathic intracranial hypertension, transverse myelitis, parkinsonian hypertonicity), the development of which is also associated with aPL synthesis. Patients with APS often have veno-occlusive eye diseases. One form of this pathology is transient loss of vision (amaurosis fugax). Another manifestation, optic neuropathy, is one of the most common causes of blindness in APS.

Cardiac disease is represented by a wide range of manifestations, including myocardial infarction, valvular heart disease, chronic ischemic cardiomyopathy, intracardiac thrombosis, arterial and pulmonary hypertension. Both in adults and children, coronary artery thrombosis is one of the main localizations of arterial occlusion in aPL overproduction. Myocardial infarction develops in approximately 5% of aPL-positive patients, and it usually occurs in men younger than 50 years. The most common cardiac sign of APS is valvular heart disease. It ranges from minimal abnormalities detected only by echocardiography (slight regurgitation, thickening of the valve leaflets) to heart disease (stenosis or insufficiency of the mitral, less often aortic and tricuspid valves). Despite the large distribution, clinically significant pathology leading to heart failure and requiring surgical treatment, rarely observed (in 5% of patients). However, in some cases, very severe damage to the valves with vegetations caused by thrombotic layers, indistinguishable from infective endocarditis, can quickly develop. Detection of vegetations on the valves, especially if they are combined with hemorrhages in the subungual bed and "drum fingers", creates complex diagnostic problems and the need for a differential diagnosis with. Within the framework of AF, the development of cardiac thrombi mimicking myxoma has been described.

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

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

In APS, liver damage occurs (Budd-Chiari syndrome, nodular regenerative hyperplasia, portal hypertension), gastrointestinal tract (gastrointestinal bleeding, splenic infarction, thrombosis of mesenteric vessels), musculoskeletal system (aseptic bone necrosis).

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

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

Diagnostic criteria

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

Clinical Criteria:

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

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

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

  - three or more consecutive cases of spontaneous abortions up to 10 weeks of pregnancy (with the exclusion of anatomical defects of the uterus, hormonal disorders, maternal and paternal chromosomal disorders).

Laboratory Criteria:

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

Differential Diagnosis

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

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

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

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

APS treatment

Prevention and APS treatment present a difficult problem. This is due to the heterogeneity of pathogenetic mechanisms, polymorphism of clinical manifestations, as well as the lack of reliable clinical and laboratory indicators that allow predicting the recurrence of thrombotic disorders. There are no universally accepted international treatment standards, and the proposed recommendations are based mainly on the results of open drug trials or retrospective analysis of disease outcomes.

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

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

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

Patients with clinical signs of APS (primarily thrombosis) need more aggressive anticoagulant therapy. Treatment with vitamin K antagonists (warfarin, phenylin, acenocoumarol) is undoubtedly a more effective, but less safe (compared to ASA) method of preventing venous and arterial thrombosis. The use of vitamin K antagonists requires careful clinical and laboratory monitoring. Firstly, it is associated with an increased risk of bleeding, and the risk of developing this complication due to its severity outweighs the benefit of preventing thrombosis. Secondly, in some patients, recurrence of thrombosis is noted after discontinuation of anticoagulant therapy (especially during the first 6 months after discontinuation). Thirdly, in patients with APS, pronounced spontaneous fluctuations in the international normalized ratio (INR) can be observed, which greatly complicates the use of this indicator for monitoring warfarin treatment. However, all of the above should not be an obstacle to active anticoagulant therapy in those patients who need it ( tab. 2).

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

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

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

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

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

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

CAPS is the only absolute indication for plasmapheresis sessions, which should be combined with maximum intensive anticoagulant therapy, the use of fresh frozen plasma and pulse therapy with glucocorticoids and cytostatics. SLE and to prevent the "rebound syndrome" after plasmapheresis sessions. The use of prostacycline (5 ng / kg / min for 7 days) is justified, however, due to the possibility of developing “rebound” thrombosis, treatment should be carried out with caution.

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

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

Moderate thrombocytopenia in patients with APS does not require special treatment. In secondary APS, thrombocytopenia is well controlled with glucocorticoids, aminoquinoline drugs, and, in some cases, low doses of ASA. Tactics for the treatment of resistant thrombocytopenia, which creates a threat of bleeding, include the use of high doses of glucocorticoids and intravenous immunoglobulin. If high doses of glucocorticoids are ineffective, splenectomy is the treatment of choice.

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

All patients with APS should be under long-term dispensary observation, the primary task of which is to assess the risk of recurrent thrombosis and their prevention. It is necessary to control the activity of the underlying disease (in secondary APS), timely detection and treatment of comorbidities, including infectious complications, as well as the impact on correctable risk factors for thrombosis. It has been established that arterial thrombosis, a high frequency of thrombotic complications and thrombocytopenia are prognostically unfavorable factors in relation to lethality in APS, and the presence of lupus anticoagulant is one of the laboratory markers. The course of APS, the severity and prevalence of thrombotic complications are unpredictable; unfortunately, there are no universal treatment regimens. The above facts, as well as the multiorganism of symptoms, require the association of doctors of various specialties to solve the problems associated with the management of this category of patients.

N. G. Klyukvina, Candidate of Medical Sciences, Associate Professor
MMA them. I. M. Sechenov, Moscow

Autoimmune pathology, which is based on the formation of antibodies to phospholipids, which are the main lipid components of cell membranes. Antiphospholipid syndrome can be manifested by venous and arterial thrombosis, arterial hypertension, valvular heart disease, obstetric pathology (recurrent miscarriage, intrauterine death of the fetus, preeclampsia), skin lesions, thrombocytopenia, hemolytic anemia. The main diagnostic markers of antiphospholipid syndrome are antibodies to cardiolipin and lupus anticoagulant. Treatment of antiphospholipid syndrome is reduced to the prevention of thrombosis, the appointment of anticoagulants and antiplatelet agents.

General information

Antiphospholipid syndrome (APS) is a complex of disorders caused by an autoimmune reaction to phospholipid structures present on cell membranes. The disease was described in detail by the English rheumatologist Hughes in 1986. Data on the true prevalence of antiphospholipid syndrome are not available; it is known that insignificant levels of antibodies to phospholipids in the blood serum are found in 2-4% of practically healthy individuals, and high titers - in 0.2%. Antiphospholipid syndrome is 5 times more likely to be diagnosed among young women (20-40 years old), although men and children (including newborns) can suffer from the disease. As a multidisciplinary problem, antiphospholipid syndrome (APS) attracts the attention of specialists in the field of rheumatology, obstetrics and gynecology, and cardiology.

Causes

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

Hyperproduction of antiphospholipid antibodies can be observed with malignant neoplasms, taking medications (psychotropic drugs, hormonal contraceptives, etc.), the abolition of anticoagulants. There is evidence of a genetic predisposition to increased synthesis of antibodies to phospholipids in persons carrying HLA DR4, DR7, DRw53 antigens and in relatives of patients with antiphospholipid syndrome. In general, the immunobiological mechanisms of the development of the antiphospholipid syndrome require further study and clarification.

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

Classification

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

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

Symptoms of antiphospholipid syndrome

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

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

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

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

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

Diagnostics

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

Pregnant women with antiphospholipid syndrome need to monitor the parameters of the blood coagulation system, conduct dynamic ultrasound of the fetus and

Treatment of antiphospholipid syndrome

The main goal of antiphospholipid syndrome therapy is to prevent thromboembolic complications. Regime moments provide for moderate physical activity, the rejection of a long stay in a stationary state, traumatic sports and long air travel. Women with antiphospholipid syndrome should not be prescribed oral contraceptives, and before planning pregnancy, it is imperative to contact an obstetrician-gynecologist. Pregnant patients during the entire gestation period are shown taking small doses of glucocorticoids and antiplatelet agents, the introduction of immunoglobulin, heparin injections under the control of hemostasiogram parameters.

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

Forecast

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

In some diseases, systemic lupus erythematosus [in 70% of cases], systemic scleroderma, rheumatoid arthritis, malignant tumors, chronic infections, etc.) antibodies are produced that can attack phospholipids - components of cell membranes. Attaching to the walls of blood vessels, platelets, directly entering into blood coagulation reactions, such antibodies to phospholipids lead to the development of thrombosis.

In addition, some scientists believe that a direct "toxic" effect of this group of antibodies on body tissues is possible. The complex of symptoms manifested in this case is called antiphospholipid syndrome (APS), and in 1994 at an international symposium on antibodies to phospholipids, it was proposed to name APS Hughes syndrome(Hughes) - named after the English rheumatologist who first described it and made the greatest contribution to the study of this problem.

There are a great many antibodies to phospholipids: antibodies to cardiolipin, lupus anticoagulant, b2-glycoprotein-1-cofactor-dependent antibodies, antibodies to blood coagulation factors, antibodies to substances that, on the contrary, interfere with this process, and many, many others. In practice, the first two are usually most often determined - antibodies to cardiolipin, lupus anticoagulant.

How is it manifested?

The clinical picture in antiphospholipid syndrome can be very different and will depend on:

• the size of the affected vessels (small, medium, large);
• the speed of blockage of the vessel (slow closure of its lumen by a thrombus that has grown in it, or fast - by a detached thrombus that "migrated" into this vessel from another);
• them functional purpose(arteries or veins);
• locations (brain, lungs, heart, skin, kidneys, liver).

If small vessels are thrombosed, this leads to relatively mild dysfunction of the organ. Thus, when the small branches of the coronary arteries in the heart are blocked, the ability of individual sections of the heart muscle to contract is impaired, while the closure of the lumen of the main trunk of the coronary artery will cause the development of myocardial infarction.

With thrombosis, the symptoms often appear imperceptibly, gradually, the dysfunction of the organ increases gradually, imitating any chronic illness(liver cirrhosis, Alzheimer's disease). Blockage of the vessel by a detached thrombus, on the contrary, will lead to the development of "catastrophic disorders" of the functions of the organ. So, pulmonary embolism is manifested by attacks of suffocation, pain in chest, cough, in most cases it leads to death.

Antiphospholipid syndrome can mimic a variety of diseases, but some symptoms are worth paying special attention to.

Quite often, with antiphospholipid syndrome, there are livedo reticularis (lacy, thin mesh of blood vessels on the surface of the skin, which becomes better visible in the cold), chronic leg ulcers that are difficult to treat, peripheral gangrene (necrosis of the skin or even individual fingers or toes).

In men, more often than in women, a manifestation of antiphospholipid syndrome may be myocardial infarction.

In women, it is more often cerebral circulation(stroke, especially before the age of 40, migraine-like headaches).

Damage to the vessels of the liver can lead to an increase in its size, ascites (accumulation of fluid in the abdominal cavity), an increase in the concentration of liver enzymes (aspartate and alanine aminotransferase) in the blood. If the vessels of the kidneys are affected, arterial hypertension develops (in this regard, people who which pressure, especially lower, high, often changes during the day).

With thrombosis of the arteries of the placenta, intrauterine fetal death may occur or premature birth. It is precisely with the antiphospholipid syndrome that women with systemic lupus erythematosus cannot "save" their pregnancy, which often ends in miscarriage.

How to suspect?

The presence of antiphospholipid syndrome can be suspected in the following cases:

• If a person has systemic lupus erythematosus (the incidence of antiphospholipid syndrome in this disease is extremely high).
• If a person under the age of 40 shows signs of thrombosis of any vessels.
• If vessels are thrombosed, for which this is not very typical, for example, vessels supplying the intestines. Their blockage leads to "abdominal toad". Such a colorful name for this disease arose by analogy with angina pectoris - "angina pectoris". "Abdominal toad" is characterized by the appearance of pressing, squeezing pain in the abdomen that occurs after a heavy meal. How more people eaten, the more blood the digestive tract needs to digest food. If the lumen of the vessels is narrowed by a thrombus, then there is not enough blood to the abdominal organs, they lack oxygen, metabolic products accumulate in them - pain appears.
• If the number of platelets in the blood is reduced and there is no hematological disease.
• If a woman has had 2 or more miscarriages, and gynecologists cannot accurately determine their cause.
• If a myocardial infarction occurs in a person younger than 40 years.

Treatment

First of all, antiphospholipid syndrome is treated only under the supervision of a rheumatologist.

If the antiphospholipid syndrome has developed against the background of an autoimmune disease (for example, systemic lupus erythematosus), this disease should be treated, trying to reduce its activity. If this can be achieved, the amount of antibodies to phospholipids in the blood serum will decrease. The lower their content in the blood, the less the likelihood of thrombosis. Therefore, it is so important for the patient to take the basic therapy prescribed by the doctor (glucocorticoids, cytostatics).

With a very high titer (quantity, concentration) of antibodies, the question of plasmapheresis (blood purification) may arise.

Perhaps the doctor will prescribe any drugs that will reduce the likelihood of thrombosis by acting directly on the blood coagulation system. For their appointment, strict indications are needed: the benefits must significantly exceed side effects. Contraindications to taking these drugs are pregnancy (may cause a violation of the development of the nervous system in the fetus) and peptic ulcers of the gastrointestinal tract. You should weigh the pros and cons if the patient has liver or kidney damage.

Antimalarial drugs (eg, hydroxychloroquine) combine an anti-inflammatory effect with the ability to inhibit platelet aggregation (clumping), which also helps prevent the development of thrombosis.

Women with antiphospholipid syndrome should delay pregnancy until laboratory values ​​return to normal. If the syndrome has developed after conception, then you should think about the introduction of immunoglobulin or small doses of heparin.

The prognosis will largely depend on the timeliness of the treatment started and the discipline of the patient.

Antiphospholipid syndrome belongs to the group of rheumatological pathologies. It manifests itself in increased blood clotting and, as a result, the formation of blood clots in blood vessels (arteries and veins). The main cause of thrombus formation is the inhibition of phospholipids.

According to medical statistics, pathology most often affects women of childbearing age, the male half of the population is less susceptible to it. As for the age range, the average age of patients is from 20 to 45 years. In children and the elderly, this disease is much less common.

Causes of antiphospholipid syndrome

The syndrome can occur both independently and be provoked by other diseases. Especially often it occurs as a result of autoimmune pathologies. These include:

• systemic lupus erythematosus;
• arthritis;

Arthritis can lead to this disease

• rheumatism;
• malignant tumors;
• hepatitis A, B, C.

It can also be any infection, both bacterial and viral. Such diseases lead to a change in blood viscosity, the formation of clots that attach to the wall of a blood vessel, partially or completely clog its lumen. As a result of this process, normal blood flow through the blood vessels will be disrupted. Scientists studying antiphospholipid syndrome provide evidence that this condition can lead to the most serious consequences: heart attack, stroke, death.

In addition to the primary and secondary pathological process of inhibition of phospholipids, doctors also distinguish a catastrophic form. In this case, the prognosis for a cure is very disappointing.

This is a relatively recently discovered disease. The antiphospholipid syndrome (APS) as an independent pathology was recognized in 1994, although it had been studied for 2 decades before.

Rheumatism often leads to this pathology

Diagnosis of antiphospholipid syndrome

It is possible to identify this pathological state of the body only as a result of clinical trial blood from a vein for the presence of antibodies. The analysis should be performed twice, after a certain period of time. As a rule, the standard period for re-analysis is 3 months. After that, the doctor can already accurately confirm or refute the diagnosis and prescribe the appropriate treatment.

Indications for diagnosis will be a number of symptoms detected during an external examination or as a result of the appointment of a cardiologist, gynecologist, phlebologist and other specialists.

Symptoms of antiphospholipid syndrome

The disease has a whole range of clinical manifestations, which are determined by the localization of the process, the nature of the lesions. It can be thrombosis of capillaries, veins and arteries of any organ or system human body.

In 80% of cases, it is the venous part that is affected. circulatory system. Consequently, the symptoms can be both from the side of the cardiovascular and the central, autonomic nervous systems.

On the part of gynecology, problems with conception and gestation are possible.

The formation of varicose veins and vascular network indicates the presence of this syndrome

Dermatological disorders are manifested in the form of:

• blockage;
• the formation of varicose veins and vascular network;
• tendency to bruise for no apparent reason.

With this disease, almost the entire vascular system of the body can fail, although more often there is a localization of the pathology:

• on the retina of the fundus;
• in the vessels of the liver;
• in the veins of the extremities (often lower).

Due to the antiphospholipid syndrome, many diseases of the cardiovascular systems occur. For example: thrombophlebitis, hemorrhoids, problems with the valves of the heart muscle, varicose veins veins.

This syndrome often results in varicose veins.

Treatment of antiphospholipid syndrome

This disease requires an integrated approach under the guidance of an appropriate specialist. In this case, it is a rheumatologist and immunologist. The general treatment regimen should include the following measures:

• Preventive measures of vascular thrombosis.
• Improvement of biochemical and physical parameters of blood. In this case, it is necessary to eliminate or significantly reduce the number (titers) of antibodies to phospholipids.
• Antiplatelet agents. This is a group of drugs that improves blood circulation, preventing its coagulability.

When provoked or secondary syndrome APS main efforts should be aimed at eliminating the main cause of the developed process. The doctor prescribes medications of the appropriate direction. Thus, they achieve a decrease in the level of antibodies in the blood.

Medical therapy can be different. Most often, these are hormonal and anti-inflammatory drugs that reduce the level of phospholipid antagonists, therefore, thrombosis decreases.

Medical treatment

According to the indications, treatment aimed at reducing blood viscosity is possible. Such drugs have a lot of contraindications. On the other hand, if there are no organic disorders in the activity of the stomach, intestines, liver and kidneys, then such drugs have a good antithrombotic effect.

The doctor may also recommend drastic measures. There is a special technology of blood purification - plasmapheresis. This is enough effective procedure at very high levels of antibodies, although its use is considered exclusive for antiphospholipid syndrome.

The disease is complex, often with a chronic course, periodic relapses are possible, but with timely treatment and attentive attitude to one's health, the prognosis is very favorable. Together with the doctor, you can achieve, if not a complete recovery, then a stable remission.

Antiphospholipid syndrome and pregnancy

Unfortunately, these two conditions of the body are practically mutually exclusive concepts, since pathology leads to problems with gestation. Due to impaired blood supply to the fetal matter, there is a possibility of:

• stillbirth or miscarriage;
• embryonic infarction;

• delayed fetal development, poor weight gain.

Only after successful treatment and normalization of clinical blood counts can we talk about the normal course of pregnancy and the birth of a healthy baby. Therefore, if you are planning to have children, it is advisable to undergo a preliminary diagnosis. Especially, this is necessary if there were spontaneous miscarriages. Perhaps APS is their cause.

Be attentive to your health and carefully follow the doctor's recommendations, only in this case you can talk about a long, full and healthy life of your body.

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Antiphospholipid syndrome (APS) or antiphospholipid antibody syndrome (SAPA) four decades ago was not known even to physicians who do not deal with this problem, not to mention patients. They started talking about it only from the beginning of the 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 the same name - Hughes syndrome (some authors call it Hughes syndrome, which is probably also correct).

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

The basis of the development of the syndrome of phospholipid antibodies

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

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

Thus, if there are different classes of phospholipids, then antibodies to them will also represent a rather diverse community. Antiphospholipid antibodies (AFLA) should be of different directions, have the ability to react with certain determinants (either anionic or neutral). The most well-known, 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 Wasserman reaction. As a rule, these antibodies are immunoglobulins of classes A, G, M;
• AT manifesting itself in a 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 refers to natural anticoagulants, that is, to substances that prevent unnecessary 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 the diagnosis of the syndrome, because in itself it is associated with certain difficulties.

Diagnosis of antiphospholipid syndrome

Of course, antiphospholipid syndrome can be suspected for a number of reasons. clinical symptoms, however, the final diagnosis must be established on the basis of a combination of symptoms and immunological examination of the patient, which implies a certain (and rather broad) list of laboratory tests. These are traditional methods: general (with platelet count) and biochemical analysis blood, including a coagulogram, and specific tests aimed at detecting antibodies to phospholipids.

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

The most important methods of laboratory diagnostics 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 spectrum of symptoms of this pathology is extremely wide. It affects various fields of medicine, thus 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. Thrombi formed there, breaking away, are sent to the vessels of the lungs, clog them, and this entails the occurrence of such a dangerous, and often fatal, condition, how . Everything here depends on the size of the incoming thrombus and the caliber of the vessel in which this thrombus is stuck. If the main trunk of the pulmonary artery (LA) is closed, then it is not necessary to count on a favorable outcome - reflex cardiac arrest leads to instant death of a person. Cases of blockage of small LA branches give chances for survival, but do not exclude hemorrhages, pulmonary hypertension, pulmonary infarction and the development of heart failure, which also does not “draw” particularly bright prospects.

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

Although less common, there are thrombosis of the subclavian veins or retinal vessels, 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 of occurrence.

Arterial thromboses give the phenomena of ischemia with the development of necrosis. In a word, heart attacks, aortic arch syndrome, gangrene, aseptic necrosis of the femoral head - all this is 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 especially difficult tasks assigned to obstetrics, because a third of women who are in anticipation of the happiness of motherhood, instead receive tears and disappointments. In general, one can say that obstetric pathology has absorbed the most characteristic, but rather dangerous features of the antiphospholipid antibody syndrome:

• Miscarriage that becomes habitual;
• Recurrent spontaneous miscarriages (1 trimester), the risk of which increases in proportion to the increase in class G immunoglobulins to 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 the risk of preeclampsia, eclampsia;
• Chorea;
• Thrombosis (both in the veins and in the arteries), recurring again and again;
• Hypertension of pregnant women;
• early start and severe course diseases;
• hellp-syndrome - a dangerous pathology of the 3rd trimester (35 weeks and beyond), emergency in obstetric practice (rapid increase in symptoms: vomiting, epigastric pain, headache, swelling);
• Early, untimely separation of the placenta;
• Childbirth up to 34 weeks;
• Unsuccessful IVF attempts.

Development start pathological changes during pregnancy, of course, give vascular thrombosis, placental ischemia, placental insufficiency.

Important - don't miss it!

Women with a similar pathology during the gestation period require special attention and dynamic monitoring. The doctor who leads her knows what can threaten the pregnant woman and what she risks, so he prescribes additional examinations:

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

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, various 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 shown to take glucocorticosteroids and antiplatelet agents (in small dosages prescribed by a doctor!). Immunoglobulins and heparin are also shown, but they are used only under the control of a coagulogram.

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

Influence of AFLA on organs and systems

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

• convulsive syndrome;
• Dementia, steadily progressing and driving the patient's brain into a "plant" state;
• Various (and often very unpleasant) mental disorders.

In addition, with the syndrome of antiphospholipid antibodies, you can also find other neurological symptoms:

1. Headaches resembling those of a migraine;
2. Random involuntary movements of the limbs, characteristic of chorea;
3. Pathological processes in the spinal cord, leading to motor, sensory and pelvic disorders, coinciding in the clinic with transverse myelitis.

Heart pathology, due to 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 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 do without impaired contractility of the heart muscle. APS "takes an active part" in the formation of valvular defects, more rare cases- promotes formation of intracardiac thrombi and misdiagnoses as doctors begin to suspect cardiac myxoma.

APS can cause a lot of trouble to other authorities:

The variety of symptoms indicating damage to a particular organ often allows the antiphospholipid syndrome to proceed in different forms, in the form pseudosyndromes mimicking another pathology. Often it behaves like a vasculitis, sometimes it manifests itself as a debut multiple sclerosis, in some cases, doctors begin to suspect a heart tumor, in others - nephritis or hepatitis ...

And a little about the treatment ...

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

1. Do not lift weights, physical activity is feasible, moderate;
2. Prolonged stay in a motionless position is unacceptable;
3. Sports activities, even with a minimal risk of injury, are highly undesirable;
4. Air travel for a long time - strongly not recommended, short trips - agreed with the doctor.

Treatment 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 in general until the end of their lives.

The prognosis for APS is not so bad if you follow all the recommendations of doctors. 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 quality life without exacerbations, as well as for a favorable pregnancy and safe delivery.

Difficulties in the prognostic plan are such unfavorable factors as the combination of ASF + SLE, thrombocytopenia, persistent arterial hypertension, and a rapid increase in antibody titers to the cardiolipin antigen. Here one can only sigh heavily: “The ways of the Lord are inscrutable…”. But this does not mean at all that the patient has so few chances ...

All patients with an updated diagnosis of "Antiphospholipid syndrome" are registered with a rheumatologist who monitors the course of the process, periodically prescribes tests (, serological markers), conducts 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 AFLA usually does not show high results. At the same time, one cannot also say that they are not detected at all in this category of citizens. Up to 12% of the examined people can have antibodies to phospholipids in their blood, but at the same time do not get sick. By the way, with age, the frequency of detection of these immunoglobulins is likely to increase, which is considered quite a natural phenomenon.

And yet, sometimes there are cases that make some especially impressionable people to be pretty worried or even go through a shock. For example, a person went to some kind of examination, which involves conducting many laboratory tests, including an analysis for syphilis. And the test turns out to be positive... Then, of course, they will double-check 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 not to panic prematurely, but also not to calm down completely, because antiphospholipid antibodies can someday remind of themselves.

Video: lectures on APS

general information

About the diagnosis of APS

APS and other thrombophilias in obstetrics