Antiphospholipid syndrome - a set of clinical and laboratory disturbances, have a tendency to increased thrombosis, thrombocytopenia and systemic hematological disorders caused by changes in the autoimmune nature.
main risk category for antiphospholipid syndrome are women during pregnancy, and the incidence of symptoms in this category of patients is at least 5%.Reliably establish the statistics of occurrence of this disease among the entire adult population is not possible, since in some cases, absolutely healthy person may experience performance frontier of antiphospholipid antibodies in the circulation.
In the absence of timely laboratory diagnostic and therapeutic measures, the conditions for significant health problems of the patient, and even a threat to the life of the patient, so those at risk need to be systematically examined for signs of antiphospholipid syndrome.
Causes of antiphospholipid syndrome
no significant etiopathogenetic factors, 100% of
Due to the fact that the latest scientific research in the field of immunology proven fact a genetic predisposition to the development of autoimmune disorders, antiphospholipid syndrome, the pathogenesis of which play an important role disorders of the immune nature, can be classified as diseases of genetic nature.
Antiphospholipid antibodies are a heterogeneous population of specific antibodies interacting with phospholipids, members of the vascular endothelium and platelets, thereby causing activation of platelet cells, loss of antithrombogenic properties of vascular endothelium and violation of fibrinolytic process.
Thus, the basis of the pathogenesis of antiphospholipid syndrome is persistent activation of the hemostatic system due to increased prothrombotic processes with simultaneous weakening of anti-thrombotic processes in the body, which inevitably leads to a recurrence of thrombosis.
According to pathogenetic theory, signs of antiphospholipid syndrome result from direct amplification processes hypercoagulable under the influence of circulating antiphospholipid antibodies ("first strike"), followed by the influence of the local of trigger mechanisms as factors induction of thrombosis (the "second strike").
In a situation where it is impossible to reliably establish the relationship between the development of autoimmune disorders with any causative factor, the conclusion is established "primary antiphospholipid syndrome."
risk for this nosology form is represented by patients, long located on bed rest during the postoperative period, women receiving long-term complex hormonal contraceptives, as well as all persons with symptoms of high cholesterol.
Symptoms of antiphospholipid syndrome
most difficult and at the same time the most frequent clinical manifestations of antiphospholipid syndrome are vascular thrombosis of various localization and severe obstetric pathology.Manifestations of thrombosis can be very different depending on the prevalence of pathologic process and size of the affected vessel.In the international surgical practice there is a perception that the antiphospholipid syndrome develop multiple noninflammatory vasculopathy nature, accompanied by the occlusion of the vessel lumen.
debut thrombotic manifestations of antiphospholipid syndrome usually occurs in lesions of deep vein distal parts of the lower extremities, but in some cases can be observed signs of vascular lesions of the arterial bed (loss of all kinds of sensitivity, cold skin, trophic changes of the skin and subcutaneous tissue).Favourite localization of arterial thrombosis in the antiphospholipid syndrome are intracerebral artery, in the event that has severe cerebral neurological symptoms.
With the defeat of the structures of the central nervous system caused by ischemic disorders, there are varying degrees of neurological manifestations of transient ischemic attack before the onset of persistent neuropsychiatric symptoms of ischemic stroke.With the development of a typical attack, transient ischemic attack, the patient noted a short-term loss of consciousness followed by dizziness, paresthesias, disturbance of visual function and the type of transient amnesia.In the case of recurrent transient ischemic attack flow, the conditions for the development of dementia with severe cognitive impairment.At this stage of antiphospholipid syndrome diagnosis is difficult because the clinical manifestations can simulate form senile dementia.
The most common complaint of patients with antiphospholipid syndrome are frequent episodes of migraine headaches accompanied by transient loss of vision.
Hyperproduction antiphospholipid antibodies promotes thrombosis of vessels various sites, including the coronary arteries, which is equally common among persons of different age categories, including children.In the elderly these changes will inevitably lead to the development of clinical symptom of myocardial infarction.Young patients with laboratory evidence of antiphospholipid syndrome, are more susceptible to changes in the appearance of valvular heart disease, hemodynamic disturbances manifest different depths.
Due to the fact that the thrombotic manifestations of antiphospholipid syndrome are observed in almost all organs of the human body, symptoms of kidney damage is severe proteinuria and clinical development of nephrotic syndrome.
thrombosis of small capillary vessels with localization in the skin is accompanied by a characteristic hemorrhagic rash and trophic disorders of the skin.
defeat of the gastrointestinal tract is increased tendency to gastric bleeding and Development Clinic mesenteric thrombosis (acute abdominal pain without clear localization, disorder chair, uncontrollable vomiting, positive symptoms of irritation of the peritoneum).
Antiphospholipid syndrome in pregnancy
Unfortunately, late diagnosis and lack of therapeutic measures aimed at addressing the symptoms of antiphospholipid syndrome in a pregnant woman can cause severe complications, both during gestation and at delivery.In some situations, the conclusion of "antiphospholipid syndrome" is established only after several episodes of premature termination of pregnancy at different stages.
current statistics, according to which even a perfectly healthy pregnant women in 2% of cases observed transient episodes enhance antiphospholipid antibodies resulting from immune disorders and require dynamic laboratory testing without medical correction.
Clinical manifestations and the degree of their manifestations depend not only on laboratory titer of antiphospholipid antibodies, as the duration of pregnancy, during which they appeared.Thus, in the first trimester of pregnancy, the presence of antibodies to phospholipids can be a provocateur occurrence of spontaneous abortion.The second trimester of pregnancy in women with antiphospholipid syndrome, occurs with signs of intrauterine growth retardation and pre-eclampsia episodes.The third trimester is dangerous in relation to the development of severe complications such as intrauterine fetal death or premature birth occurred as.
All these pathological conditions are explained by the influence of antiphospholipid antibodies thrombotic vascular placental system that provides prenatal nutrition fruit.In addition, the antiphospholipid syndrome during pregnancy triggers a cascade of hormonal changes in the body of a pregnant woman, are important in the regulation of fetal development.
There is a clear relationship between high levels of antiphospholipid antibodies in the blood of women of reproductive age and the inability to conceive a child, due to a violation of the process of implantation of the blastocyst.
Due to the fact that the antiphospholipid syndrome refers to a category of diseases that provoke severe obstetric pathology, its diagnosis must be included in the algorithm of mandatory surveys of women suffering from infertility and habitual miscarriage.
In the case of laboratory-confirmed antiphospholipid syndrome, all patients before pregnancy is recommended the use of drugs in the minimum number of glucocorticoid dosage (prednisolone at a daily dose of 5 mg) under constant control titer antibodies to phospholipids and coagulation parameters.In a situation of protracted course of antiphospholipid syndrome, a pregnant woman is forced to take all of corticosteroids during pregnancy, and even the early postpartum period.Long-term hormone therapy should be carried out "under the guise" of immunoglobulins in a dose rate of 25 ml for three days in order to prevent a possible recurrence of acute viral infection.
To minimize adverse events used for therapeutic purposes in antiphospholipid syndrome drugs, recommended a thorough medical examination and preparation of the patient during pregnancy planning.
Minimum diagnostic measures during pregnancy is to assess the degree of development of the fetus by ultrasound with a frequency of at least 1 time per month, Doppler to exclude pathology placental circulation, and in the third trimester regular CTG.
laboratory tests for antiphospholipid syndrome was first performed at the sixth week of pregnancy and just before birth.In addition, patients with antiphospholipid syndrome must be systematically carried out a study coagulation indicators, including early postpartum period in which there is a high risk of thromboembolic complications.
With existing coagulation changes, testifying in favor of the activation process of thrombus formation, to all pregnant and postpartum recommended continuation of anticoagulant therapy (heparin dose of 10 000 IU sc), which is an absolute contraindication for lactation.
Diagnosis of antiphospholipid syndrome
Due to the fact that the antiphospholipid syndrome have a variety of clinical manifestations that mimic other diseases, to establish a definite diagnosis the patient must carry out a complete laboratory and instrumental studies.
specific laboratory analysis in antiphospholipid syndrome is the study of venous blood for the presence of specific markers, which include lupus anticoagulant and anti-cardiolipin.This analysis does not have absolute certainty, even in a healthy person may be a false positive result, so it is necessary to confirm the diagnosis analysis of the product three times at intervals of three months.
In addition, non-specific analyzes in a detailed analysis of coagulation and blood count with platelet count, allow us to estimate the severity of the antiphospholipid syndrome, as well as control over the ongoing therapeutic measures.
Instrumental methods of diagnosis (magnetic resonance imaging, computed tomography, echocardioscopy) to evaluate the presence of thrombotic changes in the blood vessels and ischemic changes in the organs.
To facilitate the diagnosis "antiphospholipid syndrome", established a uniform system of criteria for the diagnosis, including clinical and laboratory criteria for antiphospholipid syndrome.In a situation where the patient has at least one of the clinical criteria combined with laboratory parameters, the diagnosis is considered to be justified.
main clinical criteria of this disease is considered to be a single dose or recurrent episode of thrombosis with different length and location.To obstetric clinical signs of antiphospholipid syndrome include: single or multiple spontaneous miscarriage in which the fetus has no developmental abnormalities, frequent bouts of episodes of preeclampsia and premature births occurring, fading in the absence of fetal chromosomal abnormalities in the parents.
Absolute laboratory criteria is the presence of high titer anticardiolipin antibodies group M, the increased concentration of antibodies to beta-glycoprotein, and lupus anticoagulant twice within three months.
Treatment of antiphospholipid syndrome
Despite the trend to an increase in the incidence of pathologies associated with antiphospholipid syndrome, has not yet developed common international standards for the monitoring and treatment of patients suffering from some form of the syndrome.However, after numerous studies involving a large group of patients, it was possible to conduct a retrospective analysis of options for a possible outcome of the disease after the application of a particular method of treatment.
There are a variety of combination therapy antiphospholipid syndrome, but the most effective in respect of correction of autoimmune disorders have the following combination of drugs: anticoagulant direct and glucocorticosteroids acetylsalicylic acid in combination with corticosteroids, isolated acetylsalicylic acid and intravenous immunoglobulin maximum dosage.
When using corticosteroid therapy is recommended the use of long-acting formulations minimal dosage to avoid potential adverse reactions from the digestive tract.
according randomized study, the combination of minimal doses of heparin (10,000 IU subcutaneous per day) with acetylsalicylic acid at a dose of 75 mg has a better effect than antiplatelet monotherapy.In a situation where a pregnant woman in addition to the laboratory changes specific to antiphospholipid syndrome, experienced at least one episode of preeclampsia, it is advisable to supplement the anticoagulant therapy of immunoglobulins in a daily dose of 400 mg per 1 kg of patient weight.
order to eliminate microcirculatory and vascular-platelet disorders Dipyridamole recommended the use of a daily dose of 150 mg, which can be used during early pregnancy.
during active anticoagulant therapy should be preferred low molecular weight heparin (Enoksiparin a daily dose of 20 mg), which can be used for a long period of time without fear of adverse reactions in the form of osteoporosis and geparinindutsirovannoy thrombocytopenia.The scope of their application are considered priority cases of antiphospholipid syndrome in pregnant women, as the active substance does not penetrate the placenta and has no adverse effects on the fetus.
prove effective against thrombotic disorders correction and elimination of autoimmune disorders has plasmapheresis.