Anti-phospholipidic syndrome - autoimmune pathology which cornerstone formation of antibodies to the phospholipids which are the main lipidic components of cellular membranes is. The anti-phospholipidic syndrome can be shown by venous and arterial thromboses, arterial hypertension, valvate heart diseases, obstetric pathology (habitual not incubation of pregnancy, pre-natal death of a fruit, gestozy), damage of skin, thrombocytopenia, hemolytic anemia. The main diagnostic markers of an anti-phospholipidic syndrome are At to a kardiolipin and volchanochny anticoagulant. Treatment of an anti-phospholipidic syndrome comes down to prevention of a tromboobrazovaniye, purpose of anticoagulants and antiagregant.
Anti-phospholipidic syndrome (AFS) – a complex of the violations caused by autoimmune reaction to the phospholipidic structures which are present at cellular membranes. The disease was in details described by the English rheumatologist of Hughes in 1986. Data on true prevalence of an anti-phospholipidic syndrome are absent; it is known that the AT insignificant levels to phospholipids in serum of blood are found in 2-4% of almost healthy faces, and high credits – in 0,2%. The anti-phospholipidic syndrome 5 times more often is diagnosed among women of young age (20-40 years) though men and children can have a disease (including newborns). As the multidisciplinary problem, an anti-phospholipidic syndrome (AFS) draws attention of experts in the field of rheumatology, obstetrics and gynecology, neurology, cardiology.
Reasons of an anti-phospholipidic syndrome
The fundamental reasons of development of an anti-phospholipidic syndrome are unknown. Meanwhile, the factors contributing to increase in level of antibodies to phospholipids are studied and defined. So, tranzitorny increase of anti-phospholipidic antibodies is observed against the background of viral and bacterial infections (hepatitis C, HIV, an infectious mononukleoz, malaria, an infectious endocarditis, etc.). High credits of antibodies to phospholipids are found in patients with a system red volchanka, rheumatoid arthritis, Shegren's disease, a sklerodermiya, a nodular periarteriit, autoimmune trombotsitopenichesky purpury.
Hyperproduction of anti-phospholipidic antibodies can be noted at malignant new growths, reception of medicines (psychotropic drugs, hormonal contraceptives, etc.), cancellation of anticoagulants. There are data on genetic predisposition to the increased synthesis of antibodies to phospholipids for persons carriers of anti-genes of HLA DR4, DR7, DRw53 and for relatives of patients with an anti-phospholipidic syndrome. In general immunobiological mechanisms of development of an anti-phospholipidic syndrome demand further studying and specification.
Depending on structure and an immunogenicity distinguish "neutral" (, phosphatidylethanolamine) and "negatively charged" (, , ) phospholipids. To a class of the anti-phospholipidic antibodies reacting with phospholipids volchanochny anticoagulant, At belong to a kardiolipin, beta2-glycoprotein-1-kofaktorzavisimye anti-phospholipids, etc. Interacting with phospholipids of membranes of cages an endoteliya of vessels, platelets, neutrophils, antibodies cause violation of a hemostasis, expressed in tendency to hyper coagulation.
Classification of an anti-phospholipidic syndrome
Taking into account an etiopatogenez and a current distinguish the following clinical laboratory options of an anti-phospholipidic syndrome:
- primary – communication with any background disease capable to induce formation of anti-phospholipidic antibodies is absent;
- secondary - an anti-phospholipidic syndrome develops against the background of other autoimmune pathology;
- catastrophic – the sharp koagulopatiya proceeding with multiple thromboses of internals;
- AFL-negativny option of an anti-phospholipidic syndrome at which serological markers of a disease (At to a kardiolipin and volchanochny anticoagulant) are not defined.
Symptoms of an anti-phospholipidic syndrome
According to modern views, the anti-phospholipidic syndrome represents an autoimmune trombotichesky vaskulopatiya. At AFS defeat can affect vessels of various caliber and localization (capillaries, large venous and arterial trunks) that causes extremely various range of clinical manifestations including venous and arterial thromboses, obstetric pathology, neurologic, cardiovascular, skin violations, thrombocytopenia.
The most frequent and typical sign of an anti-phospholipidic syndrome are recurrent venous thromboses: thrombosis of superficial and deep veins of the lower extremities, hepatic veins, vorotny vein of a liver, retina veins. Patients with an anti-phospholipidic syndrome can have repeated episodes of TELA, pulmonary hypertensia, a syndrome of the top hollow vein, Badda-Kiari's syndrome, nadpochechnikovy insufficiency. Venous thromboses at an anti-phospholipidic syndrome develop twice more often than arterial. Among the last the thromboses of cerebral arteries leading to the tranzitorny ischemic attacks and an ischemic stroke prevail. Other neurologic violations can include migraine, giperkineza, a convulsive syndrome, neurotouch relative deafness, ischemic neuropathy of an optic nerve, cross miyelit, dementia, mental violations.
Defeat of cardiovascular system at an anti-phospholipidic syndrome is followed by development of a myocardial infarction, intracardial thrombosis, an ischemic cardiomyopathy, arterial hypertension. Defeat of valves of heart – from the insignificant regurgitation revealed by means of EhoKG to a mitralny, aortal, trikuspidalny stenosis or insufficiency is quite often noted. Within diagnostics of an anti-phospholipidic syndrome with warm manifestations performing differential diagnostics with an infectious endocarditis, miksomy hearts is required.
Kidney manifestations can include both an insignificant proteinuria, and a sharp renal failure. From bodies of a GIT at an anti-phospholipidic syndrome the gepatomegaliya, gastrointestinal bleedings, occlusion of mezenterialny vessels, portal hypertensia, a spleen heart attack meet. Typical damages of skin and soft fabrics are presented mesh , palmar and plantar eritemy, by trophic ulcers, gangrene of fingers; the musculoskeletal device - aseptic necroses of bones (a femur head). Thrombocytopenia, hemolytic anemia, hemorrhagic complications are hematologic signs of an anti-phospholipidic syndrome.
At women of AFS often comes to light in connection with obstetric pathology: repeated spontaneous termination of pregnancy in various terms, a delay of pre-natal development of a fruit, fetoplatsentarny insufficiency, gestozy, a chronic hypoxia of a fruit, premature birth. When conducting pregnancy at women with an anti-phospholipidic syndrome the obstetrician-gynecologist has to consider all possible risks.
Diagnostics of an anti-phospholipidic syndrome
The anti-phospholipidic syndrome is diagnosed on the basis of clinical (vascular thrombosis, the burdened obstetric anamnesis) and laboratory data. The main immunological criteria include identification in plasma of blood of average or high credits of At to a kardiolipin of the class IgG/IgM and volchanochny anticoagulant twice within six weeks. The diagnosis is considered reliable at a combination of, at least, one main clinical and laboratory criterion. Additional laboratory signs of an anti-phospholipidic syndrome are false positive RW, positive reaction of Koombs, increase in a caption of an anti-nuclear factor, rheumatoid factor, cryoglobulins, antibodies to DNA. Research OAK, platelets, biochemical blood test, a koagulogramma is also shown.
Pregnant women with an anti-phospholipidic syndrome need monitoring of indicators of the curtailing system of blood, performing dynamic ultrasonography of a fruit and doppler sonography of a uterine and placentary blood-groove, kardiotografiya. For confirmation of thromboses of internals UZDG of vessels of the head and a neck, vessels of kidneys, arteries and veins of extremities, eye vessels, etc. is carried out. Changes of shutters of heart valves come to light in the course of EhoKG.
Differential and diagnostic actions have to be directed to an exception of the DVS-syndrome, a gemolitiko-uraemic syndrome, trombotsitopenichesky purples, etc. Considering a defeat poliorgannost, diagnostics and treatment of an anti-phospholipidic syndrome demand combination of efforts of doctors of various specialties: rheumatologists, cardiologists, neurologists, obstetricians-gynecologists, etc.
Treatment of an anti-phospholipidic syndrome
As main objective of therapy of an anti-phospholipidic syndrome serves prevention of tromboembolichesky complications. The regime moments provide moderate physical activity, refusal of long stay in a motionless state, occupations traumatic sports and long air flights. Women with an anti-phospholipidic syndrome should not appoint oral contraceptives, and before planning of pregnancy it is necessary to address the obstetrician-gynecologist surely. Reception of small doses of glucocorticoids and antiagregant, introduction of immunoglobulin, a heparin injection under control of indicators of a gemostaziogramma is shown to pregnant patients during the entire period of a gestation.
Medicamentous therapy at an anti-phospholipidic syndrome can include purpose of indirect anticoagulants (varfarin), direct anticoagulants (heparin, a nadroparin of calcium, an enoksaparin of sodium), antiagregant (acetilsalicylic acid, a dipiridamol, a pentoksifillin). Preventive antikoagulyantny or antiagregantny therapy to most of patients with an anti-phospholipidic syndrome is carried out is long, and sometimes for life. At a catastrophic form of an anti-phospholipidic syndrome purpose of high doses of glucocorticoids and anticoagulants, holding sessions of a plasma exchange, transfusion of freshly frozen plasma etc. is shown.
The forecast at an anti-phospholipidic syndrome
Timely diagnostics and preventive therapy allow to avoid development and a retsidivirovaniye of thromboses, and also to hope for the favorable result of pregnancy and childbirth. At a secondary anti-phospholipidic syndrome control of the course of the main pathology, prevention of infections is represented important. Predictively as adverse factors serve the combination of an anti-phospholipidic syndrome to hard currency, thrombocytopenia, bystry increase of a caption of At to a kardiolipin, persistent arterial hypertension. All patients with the diagnosis "an anti-phospholipidic syndrome" have to be under observation of the rheumatologist with periodic control of serological markers of a disease and indicators of a gemostaziogramma.