Phospholipid syndrome: causes and diagnostics

Phospholipid syndrome is a relatively common pathology of autoimmune origin. Against the background of the disease often there are lesions of blood vessels, kidneys, bones and other organs. In the absence of therapy, the ailment can lead to dangerous complications until the patient dies. Moreover, the disease is often found in women during pregnancy, which jeopardizes the health of the mother and child.

Of course, many people are looking for additional information, asking questions about the causes of the disease. What symptoms should I pay attention to? Is there an analysis for phospholipid syndrome? Can medicine offer effective methods of treatment?

Phospholipid syndrome: what is it?

For the first time this disease was described not so long ago. Official information about him was published in the 1980s. Since he worked on the study of English rheumatologist Graham Hughes, the ailment is often called Hughes syndrome. There are other names - antiphospholipid syndrome and the syndrome of antiphospholipid antibodies.

Phospholipid syndrome is an autoimmune disease in which the immune system begins to produce antibodies that attack the body's own phospholipids. Since these substances are part of the membrane walls of many cells, the lesions in such a disease are significant:

• Antibodies attack healthy endothelial cells, reducing the synthesis of growth factors and prostacyclin, which is responsible for the expansion of the walls of the vessels. Against the background of the disease there is a violation of platelet aggregation.
• Phospholipids are also found in the walls of the platelets themselves, which leads to an increase in their aggregation, as well as rapid destruction.
• In the presence of antibodies, there is an increase in blood coagulability and a decrease in heparin activity.
• The process of destruction does not bypass the nerve cells.

Blood begins to coagulate in the blood vessels, forming blood clots, which disrupt the blood flow, and consequently, the functions of different organs - this is how the phospholipid syndrome develops. The causes and symptoms of this ailment are of interest to many people. After all, the earlier the disease is detected, the less complications will develop in the patient.

The main causes of the development of the disease

Why do people develop phospholipid syndrome? The reasons can be different. It is known that quite often in patients there is a genetic predisposition. The disease develops in the case of an incorrect functioning of the immune system, which for one reason or another starts to produce antibodies to the cells of its own organism. In any case, the disease must be something provoked. To date, scientists have been able to identify several risk factors:

• Often the phospholipid syndrome develops against a background of microangiopathies, in particular, trobocytopenia, hemolytic-uremic syndrome.
• Risk factors include other autoimmune diseases, such as lupus erythematosus, vasculitis, scleroderma.
• The ailment often develops in the presence of malignant tumors in the patient's body.
• Risk factors include infectious diseases. Especially dangerous is infectious mononucleosis and AIDS.
• Antibodies can appear with DIC-syndrome.
• It is known that the disease can develop against the background of taking certain medications, including hormonal contraceptives, psychotropic drugs, Novokainamid, etc.

Naturally, it is important to find out why the patient developed a phospholipid syndrome. Diagnosis and treatment should identify and if possible eliminate the root cause of the disease.

Disorders of the cardiovascular system with phospholipid syndrome

Blood and blood vessels are these first "targets" that affect the phospholipid syndrome. Symptoms of it depend on the stage of the disease. Thrombi, as a rule, first formed in small vessels of the extremities. They disrupt the bloodstream, which is accompanied by tissue ischemia. The affected limb is always colder to the touch, the skin pales, and the muscles gradually atrophy. Prolonged disruption of tissue supply leads to necrosis and subsequent gangrene.

Thrombosis of the deep veins of the extremities is also possible, which is accompanied by the appearance of edema, pain, and mobility disorders. Phospholipid syndrome can be complicated by thrombophlebitis (inflammation of the vascular walls), which is accompanied by fever, chills, reddening of the skin in the affected area and acute, sharp pain.

The formation of thrombi in large vessels can lead to the development of the following pathologies:

• Aortic syndrome (accompanied by a sharp increase in pressure in the vessels of the upper body);
• Syndrome of the superior vena cava (for this condition is characterized by puffiness, cyanosis of the skin, bleeding from the nose, trachea and esophagus);
• Syndrome of the inferior vena cava (accompanied by a violation of blood circulation in the lower body, swelling of the extremities, pain in the legs, buttocks, abdomen and groin).

Thrombosis affects the work of the heart. Often the ailment is accompanied by the development of angina pectoris, persistent arterial hypertension, myocardial infarction.

Kidney damage and underlying symptoms

The formation of thrombi leads to a violation of blood circulation, not only in the extremities - the internal organs, in particular the kidneys, also suffer. With prolonged development of phospholipid syndrome, a so-called infarct of the kidney is possible. This condition is accompanied by pain in the lower back, a decrease in the amount of urine and the presence of blood impurities in it.

A thrombus can block the renal artery, which is accompanied by sharp pains, nausea and vomiting. This is a dangerous condition - in the absence of treatment, the necrotic process may develop. The dangerous effects of phospholipid syndrome include renal microangiopathy, in which small thrombi form directly in the renal glomeruli. This condition often leads to the development of chronic renal failure.

Sometimes there is a violation of blood circulation in the adrenal glands, which leads to a violation of the hormonal background.

What other organs can be affected?

Phospholipid syndrome is an ailment that affects many organs. As already mentioned, antibodies affect the membranes of nerve cells, which can not do without consequences. Many patients complain of persistent severe headaches, which are often accompanied by dizziness, nausea and vomiting. There is a possibility of developing various mental disorders.

In some patients, thrombi are found in blood vessels that supply blood to the visual analyzer. Prolonged deficiency of oxygen and nutrients leads to atrophy of the optic nerve. Possible thrombosis of the vessels of the retina with a subsequent hemorrhage. Some of the eye pathologies, unfortunately, are irreversible: visual disturbances remain with the patient for life.

In the pathological process, bones can also be involved. People often diagnosed reversible osteoporosis, which is accompanied by deformation of the skeleton and frequent fractures. Aseptic necrosis of bone is more dangerous.

For the disease is characterized by skin lesions. Often on the skin of the upper and lower extremities, vascular asterisks are formed. Sometimes you can notice a very characteristic rash, which resembles small, pinpoint hemorrhages. Some patients develop erythema on the soles of the feet and palms. There is frequent formation of subcutaneous hematomas (for no apparent reason) and hemorrhage under the nail plate. Long-term disruption of tissue trophism entails the appearance of ulcers, which for a long time heal and are difficult to treat.

We found out what the phospholipid syndrome is. Causes and symptoms of the disease are extremely important issues. After all, it is from these factors that the treatment scheme chosen by the doctor will depend.

Phospholipid syndrome: diagnosis

Of course, in this case it is extremely important to detect the presence of the disease in time. Suspect phospholipid syndrome the doctor can still during the collection of anamnesis. To suggest this idea is the patient's thrombosis and trophic ulcers, frequent miscarriages, signs of anemia. Of course, further surveys are conducted.

The analysis for phospholipid syndrome is to determine the level of antibodies to phospholipids in the blood of patients. In the general blood test, you can see a decrease in platelet count, an increase in ESR, an increase in the number of leukocytes. Often, the syndrome is accompanied by hemolytic anemia, which can also be seen during a laboratory study.

In addition, a biochemical blood test is performed. In patients, an increase in the number of gamma globulins is observed. If the liver was damaged on the background of pathology, then the amount of bilirubin and alkaline phosphatase increases in the blood . In the presence of kidney diseases, an increase in the level of creatinine and urea can be observed.

Some patients are recommended specific immunological blood tests. For example, laboratory studies can be conducted to determine the rheumatoid factor and lupus coagulant. When the phospholipid syndrome in the blood can detect the presence of antibodies to erythrocytes, increasing the level of lymphocytes. If there are suspicions of severe damage to the liver, kidneys, bones, then instrumental examinations are carried out, including X-ray, ultrasound, tomography.

What are the complications of the disease?

In the absence of treatment, phospholipid syndrome can lead to extremely dangerous complications. Against the background of the disease, clots form in the blood vessels, which is dangerous in itself. Blood clots clog vessels, disrupting normal blood circulation - tissues and organs do not receive enough nutrients and oxygen.

Often, against the background of the disease in patients develops a stroke and myocardial infarction. Blockage of the vessels of the extremities can lead to the development of gangrene. As already mentioned above, patients have abnormalities in the work of the kidneys and adrenal glands. The most dangerous consequence is thromboembolism of the pulmonary artery - this pathology develops sharply, and not in all cases the patient manages to deliver to the hospital in time.

Pregnancy in patients with phospholipid syndrome

As already mentioned, the phospholipid syndrome is diagnosed during pregnancy. What is the danger of an ailment and what to do in such a situation?

Because of the phospholipid syndrome, blood vessels form in the vessels, which clog the arteries that carry blood to the placenta. The embryo does not receive enough oxygen and nutrients, in 95% of cases it leads to miscarriage. Even if the pregnancy has not been interrupted, there is a risk of early placental abruption and the development of late gestosis, which is very dangerous for both the mother and the child.

Ideally, a woman should take tests at the planning stage. Nevertheless, phospholipid syndrome is often diagnosed during pregnancy. In such cases it is very important to notice the presence of the disease in time and take the necessary measures. For the prevention of thrombosis of the future mother can be appointed reception of anticoagulants in small doses. In addition, a woman should regularly undergo examinations so that the doctor can notice the onset of placental abruption. Every few months, future mothers undergo a course of restorative therapy, taking medications containing vitamins, minerals and antioxidants. With the right approach, pregnancy often ends up safely.

What does the treatment look like?

What to do if a person has a phospholipid syndrome? Treatment in this case is complex, and it depends on the presence of the patient of certain complications. Since blood clots form on the background of the disease, the therapy is primarily aimed at diluting the blood. The treatment regimen, as a rule, includes the use of several groups of drugs:

• First of all anticoagulants of indirect action and antiaggregants ("Aspirin", "Warfarin") are appointed.
• Often, therapy includes selective anti-inflammatory drugs of non-steroidal origin, in particular "Nimesulide" or "Celecoxib."
• If the ailment is associated with systemic lupus erythematosus and some other autoimmune diseases, the doctor may prescribe glucocorticoids (hormonal anti-inflammatory drugs). Along with this, immunosuppressive drugs can be used that suppress the activity of the immune system and reduce the production of dangerous antibodies.
• Pregnant women are sometimes given intravenous immunoglobulin.
• Patients periodically take drugs containing B vitamins.
• For general improvement, protection of blood vessels and cell membranes, antioxidant drugs are used, as well as preparations that contain a complex of polyunsaturated fatty acids (Omakor, Mexicor).

Useful on the patient's condition is the electrophoresis procedure. If it is a secondary phospholipid syndrome, it is important to control the primary disease. For example, patients with vasculitis and lupus should receive adequate treatment for these pathologies. It is also important to detect infectious diseases in time and conduct appropriate therapy until full recovery (if possible).

Projections for patients

If the phospholipid syndrome was diagnosed on time and the patient received the necessary help, then the prognosis is very favorable. It is unfortunately impossible to get rid of the disease forever, but with the help of medications one can control its exacerbations and carry out prophylactic treatment of thrombosis. Dangerous are situations in which the ailment is associated with thrombocytopenia and high blood pressure.

In any case, under the control of a rheumatologist, all patients with a diagnosis of "phospholipid syndrome" should be present. Through how many they repeat the analysis, how often it is necessary to undergo examinations with other doctors, what preparations you need to take, how to monitor the condition of your own organism - the doctor in charge will tell about all this.