Antiphospholipid Syndrome

Antiphospholipid syndrome (APS) is a symptom complex that includes the following manifestations of the disease:

Recurrent arterial and venous thromboses;

thrombocytopenia;

Various forms of obstetric pathology;

A variety of cardiovascular, hematological, neurological and other disorders.

Antiphospholipid syndrome is most often a genetic disorder. Diagnosis of antiphospholipid syndrome in the Yusupov hospital is carried out using modern laboratory methods. Serological markers of antiphospholipid syndrome are antibodies to phospholipids, cardiolipin , lupus anticoagulant, b2-glycoprotein-1-cofactor-dependent antibodies.

Types and symptoms of antiphospholipid syndrome

There are the following clinical variants of antiphospholipid syndrome:

Primary;

Secondary – with rheumatic and autoimmune diseases, malignant neoplasms, the use of drugs, infectious diseases, the presence of other causes;

Other options are “catastrophic” antiphospholipid syndrome, microangiopathic syndromes (HELLP syndrome, thrombotic thrombocytopenia, hemolytic- uremic syndrome, hypothrombinemia syndrome , disseminated intravascular coagulation), antiphospholipid syndrome in combination with vasculitis.

At the onset of the antiphospholipid syndrome, signs of damage to the cerebral vessels predominate – from memory loss, migraine, constant headache, transient visual impairment and cerebral circulation to thrombosis of the cerebral sinuses, cerebral vessels, epilepsy, thrombotic strokes and Sneddon ‘s syndrome . The first may be thrombosis of the veins of the extremities with or without pulmonary embolism, Raynaud’s syndrome .

To establish an accurate diagnosis, rheumatologists, if antiphospholipid syndrome is suspected, prescribe tests. Antibodies to phospholipids are determined in the presence of the following indications:

All patients with systemic lupus erythematosus;

Patients under the age of 40 with venous or arterial thrombosis;

With unusual localization of thrombosis (for example, in the mesenteric veins);

In case of unexplained neonatal thrombosis;

Patients with idiopathic thrombocytopenia (to exclude Moshkovich’s disease);

With the development of skin necrosis against the background of taking indirect anticoagulants;

In case of unexplained prolongation of activated partial thromboplastin time;

With recurrent spontaneous abortions;

If the patient is diagnosed with early acute myocardial infarction.

The study is performed if the patient has relatives with thrombotic disorders.

Antiphospholipid syndrome and pregnancy

In 80% of cases of antiphospholipid syndrome (APS), the manifestation of the disease is obstetric pathology (repeated episodes of antenatal fetal death, recurrent miscarriage ). Fetal loss occurs at any stage of pregnancy, but is somewhat more common in the second and third trimesters. For the treatment of pregnant women suffering from antiphospholipid syndrome, all conditions have been created at the Yusupov hospital:

Comfortable rooms;

Individual approach to the choice of drugs for the treatment of each patient;

Caring medical staff.

The production of antibodies to phospholipids is also associated with other forms of obstetric pathology: late preeclampsia, preeclampsia and eclampsia, premature birth, intrauterine growth retardation. Often, adverse outcomes are also recorded during pregnancy, which occurred against the background of severe pathology of the internal organs or is accompanied by the development of specific complications of the gestational process.

Management of pregnancy in antiphospholipid syndrome

Due to the high risk of reproductive losses at any gestational age , the management of pregnant women with antiphospholipid syndrome has some peculiarities. The risk of repeated spontaneous miscarriages up to 20 weeks of gestation when antiphospholipid antigens are detected in the blood of a pregnant woman varies from 53 to 77%. The risk of sudden fetal death in the second and third trimesters is in the range of 22-46%.

Only in 8-10% of cases, pregnancy against the background of antiphospholipid syndrome ends with the birth of a live child. Against the background of treatment with acetylsalicylic acid or heparin, almost all women give birth to live babies. Due to the high risk of adverse obstetric outcomes in antiphospholipid syndrome, doctors plan pregnancies in these patients, carefully select drug therapy, and constantly monitor the condition of the mother and fetus.

At the stage of pregnancy planning, the patient excludes concomitant risk factors for the development of thrombosis. The range of preventive measures is as follows:

Overweight correction;

Treatment of concomitant arterial hypertension;

Correction of lipid spectrum disorders.

Doctors recommend that women stop smoking and use compression stockings for post-thrombophlebitic syndrome. For patients with secondary antiphospholipid syndrome, rheumatologists at the Yusupov Hospital correct the underlying disease and individually select drugs for safe drug treatment. During pregnancy, cytostatic therapy is not carried out , as they cause abnormalities in the development of the fetus. Warfarin, which is used for anticoagulant therapy in patients with antiphospholytic syndrome, due to the possibility of developing coumarin embryopathies during pregnancy are not prescribed. If an unplanned desired pregnancy occurs while taking warfarin, the drug is canceled as soon as possible, preferably before the sixth week of gestation .

Symptoms of antiphospholipid syndrome

The following clinical manifestations of antiphospholipid syndrome are determined on the skin:

Livedo reticularis – a vascular network in the form of bluish spots on the hands, shins, thighs, hands, which is especially well detected when cooled;

Hemorrhages and heart attacks;

Thrombosis of one of the central veins;

Superficial rash in the form of pinpoint hemorrhages that resemble vasculitis.

Skin signs of antiphospholipid syndrome include necrosis of the skin of the distal lower extremities, hemorrhage in the subungual bed (splinter symptom), chronic ulcers of the extremities, palmar and plantar erythema, and skin nodules.

Patients suffering from antiphospholipid syndrome may develop deep vein thrombosis, thrombophlebitis; ischemia as a result of chronic arterial thrombosis, gangrene. Large vessels are affected with the development of the syndrome of the superior or inferior vena cava, aortic arch syndrome. When bones are damaged, aseptic necrosis develops, transient osteoporosis in the absence of glucocorticoid hormones. Renal artery thrombosis, renal infarction, intraglomerular microthrombosis with subsequent development of glomerulosclerosis and chronic renal failure.

With antiphospholipid syndrome, a clinic of damage to the organ of vision is determined. Develops thrombosis of veins, arteries and arterioles of the retina, atrophy of the optic nerve, retinal infarctions; small exudates that appear due to blockage of retinal arterioles.

A manifestation of antiphospholipid syndrome can be adrenal pathology: central vein thrombosis, heart attacks and hemorrhages, Addison’s disease, adrenal insufficiency. If the patient is receiving glucocorticoids, adrenal damage is difficult to diagnose. One of the main manifestations of antiphospholytic syndrome is obstetric pathology:

Habitual miscarriage in the absence of diseases of the female reproductive system;

Intrauterine fetal death;

Retardation of intrauterine development of the fetus;

Chorea of pregnant women;

Preeclampsia, especially its severe manifestations – preeclampsia and eclampsia;

premature birth.

If antiphospholipid syndrome is suspected, rheumatologists prescribe tests. The following laboratory criteria for the disease are known: the presence of antibodies to cardiolipin IgG or IgM in serum in medium or high titers, which are determined at least 2 times within six weeks when determined using a standardized enzyme immunoassay, and lupus antigen, which is detected in plasma at least 2 times within six weeks by a standardized method . The diagnosis of antiphospholytic syndrome is made by rheumatologists when at least one clinical and one laboratory criterion is met.

Prevention and treatment of antiphospholipid syndrome

Prevention and treatment of thrombosis in antiphospholipid syndrome is no less a challenge than its correct diagnosis. This is due to the heterogeneity of the developmental mechanisms that underlie APS, the heterogeneity of clinical manifestations, and the lack of reliable laboratory and clinical indicators that allow predicting the development of recurrent thrombotic disorders. Glucocorticoids, cytotoxic drugs and plasmapheresis in APS are used by doctors only to suppress the activity of the underlying disease or in catastrophic antiphospholytic syndrome. In other cases, they are ineffective and even contraindicated, since long-term hormonal therapy potentially increases the risk of recurrent thrombosis, and some cytotoxic drugs lead to the development of complications of anticoagulant therapy.

Due to the high risk of recurrent thrombosis, the vast majority of patients with APS for a long time, sometimes for life, undergo prophylactic anticoagulant therapy. The exception is patients with persistent normalization of the level of antibodies to phospholipids in the absence of recurrent thrombosis.

An additional preventive effect is provided by aminoquinoline drugs ( hydroxychloroquine ). It inhibits platelet aggregation and adhesion, reduces the size of a thrombus, and reduces blood lipid levels. To prevent thrombosis, patients are prescribed indirect anticoagulants, primarily warfarin. Since the use of indirect anticoagulants increases the risk of bleeding, treatment is carried out under careful laboratory and clinical control.

Treatment with warfarin can prevent recurrent venous thrombosis, but in some patients with arterial thrombosis it is not effective enough. They are given combination therapy with indirect anticoagulants and low-dose aspirin or dipyridamole . It is more justified in young people without risk factors for bleeding.

For the treatment of acute thrombotic complications in APS, direct anticoagulant heparin and low molecular weight heparin preparations are used. Treatment of catastrophic APS is carried out using the entire arsenal of methods of intensive and anti-inflammatory therapy, which is used to treat critical conditions in rheumatic diseases. Patients undergo plasmapheresis sessions, which are combined with the most intensive anticoagulant therapy, using fresh frozen plasma to replace. In the absence of contraindications, pulse therapy with glucocorticoids and cyclophosphamide is performed . Immunoglobulin ( sandoglobulin or octagama ) is administered intravenously.

How long do people live with antiphospholipid syndrome? The prognosis ultimately depends on the risk of recurrent thrombosis. In order to prevent life-threatening complications, make an appointment with a rheumatologist by calling the contact center.