Phospholipid syndrome is a relatively common pathology of autoimmune origin. Against the background of the disease, lesions of blood vessels, kidneys, bones and other organs are often observed. In the absence of therapy, the disease can lead to dangerous complications up to the death of the patient. Moreover, often the disease is detected in women during pregnancy, which endangers the he alth of mother and child.
Of course, many people seek additional information by asking questions about the causes of the development of the disease. What symptoms should you look out for? Is there an analysis for phospholipid syndrome? Can medicine offer effective treatments?
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 the English rheumatologist Graham Hughes worked on the study, the disease is often called Hughes syndrome. There are other names - antiphospholipid syndrome and antiphospholipid antibody syndrome.
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 he althy endothelial cells, reducing the synthesis of growth factors and prostacyclin, which is responsible for the expansion of the walls of blood vessels. Against the background of the disease, there is a violation of platelet aggregation.
- Phospholipids are also found in the walls of platelets themselves, which leads to increased aggregation of platelets, as well as rapid destruction.
- In the presence of antibodies, there is an increase in blood clotting and a decrease in heparin activity.
- The process of destruction does not bypass nerve cells either.
The blood begins to coagulate in the vessels, forming blood clots that disrupt the blood flow and, consequently, the functions of various organs - this is how the phospholipid syndrome develops. The causes and symptoms of this disease are of interest to many people. After all, the sooner the disease is detected, the fewer complications the patient will develop.
The main causes of the development of the disease
Why do people develop phospholipid syndrome? The reasons may be different. It is known that quite often patients have a genetic predisposition. The disease develops in case of improper functioning of the immune system, which for one reason or another begins to produce antibodies to cellsown organism. In any case, the disease must be provoked by something. To date, scientists have identified several risk factors:
- Phospholipid syndrome often develops against the background of microangiopathies, in particular trobocytopenia, hemolytic-uremic syndrome.
- Risk factors include other autoimmune diseases such as lupus erythematosus, vasculitis, scleroderma.
- The disease often develops in the presence of malignant tumors in the patient's body.
- The risk factors include infectious diseases. Of particular danger is infectious mononucleosis and AIDS.
- Antibodies may appear in DIC.
- It is known that the disease can develop while taking certain medications, including hormonal contraceptives, psychotropic drugs, Novocainamide, etc.
Naturally, it is important to find out why the patient developed phospholipid syndrome. Diagnosis and treatment should identify and, if possible, eliminate the root cause of the disease.
Losses of the cardiovascular system in phospholipid syndrome
Blood and blood vessels are the first "targets" that affect the phospholipid syndrome. Its symptoms depend on the stage of development of the disease. Thrombi usually form first in the small vessels of the extremities. They disrupt the blood flow, which is accompanied by tissue ischemia. The affected limb is always colder to the touch, the skin turns pale, and the muscles gradually atrophy. Prolonged tissue malnutrition leads to necrosis and subsequent gangrene.
Possible and deep vein thrombosis of the extremities, which is accompanied by the appearance of edema, pain, impaired mobility. 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 sharp, sharp pain.
The formation of blood clots 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 (this condition is characterized by swelling, cyanosis of the skin, bleeding from the nose, trachea and esophagus);
- inferior vena cava syndrome (accompanied by impaired circulation in the lower body, swelling of the limbs, pain in the legs, buttocks, abdomen and groin).
Thrombosis also affects the work of the heart. Often the disease is accompanied by the development of angina pectoris, persistent arterial hypertension, myocardial infarction.
Kidney damage and main symptoms
The formation of blood clots leads to circulatory disorders not only in the limbs - internal organs, in particular the kidneys, also suffer. With prolonged development of phospholipid syndrome, the so-called kidney infarction 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 severe pain, nausea and vomiting. This is a dangerous condition - if left untreated, it is possible to developnecrotic process. The dangerous consequences of the phospholipid syndrome include renal microangiopathy, in which small blood clots 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 a disease that affects many organs. As already mentioned, antibodies affect the sheaths of nerve cells, which cannot do without consequences. Many patients complain of constant severe headaches, which are often accompanied by dizziness, nausea and vomiting. There is a possibility of developing various mental disorders.
In some patients, blood clots are found in the vessels that supply the visual analyzer with blood. Prolonged deficiency of oxygen and nutrients leads to atrophy of the optic nerve. Retinal vascular thrombosis with subsequent hemorrhage is possible. Some of the eye pathologies, unfortunately, are irreversible: visual impairments remain with the patient for life.
Bone may also be involved in the pathological process. People are often diagnosed with reversible osteoporosis, which is accompanied by skeletal deformity and frequent fractures. More dangerous is aseptic bone necrosis.
Skin lesions are also characteristic of the disease. Often, spider veins form on the skin of the upper and lower extremities. Sometimes you can notice a very characteristic rash that 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 hemorrhages under the nail plate. A long-term violation of tissue trophism leads to the appearance of ulcers that take a long time to heal and are difficult to treat.
We figured out what phospholipid syndrome is. The causes and symptoms of the disease are extremely important questions. After all, the treatment regimen chosen by the doctor will depend on these factors.
Phospholipid syndrome: diagnosis
Of course, in this case it is extremely important to detect the presence of the disease in time. A doctor can suspect phospholipid syndrome even during the collection of anamnesis. The presence of thrombosis and trophic ulcers in the patient, frequent miscarriages, signs of anemia can lead to this thought. Of course, additional examinations are carried out in the future.
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 notice a decrease in the level of platelets, 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.
Additionally, a biochemical blood test is performed. Patients have an increase in the amount of gamma globulins. If the liver was damaged against the background of the pathology, then the amount ofbilirubin and alkaline phosphatase. In the presence of kidney disease, an increase in the level of creatinine and urea can be observed.
Some patients are also recommended specific immunological blood tests. For example, laboratory tests may be performed to determine rheumatoid factor and lupus coagulant. With phospholipid syndrome in the blood, the presence of antibodies to erythrocytes, an increase in the level of lymphocytes can be detected. If there are suspicions of severe damage to the liver, kidneys, bones, then instrumental examinations are performed, including X-ray, ultrasound, tomography.
What are the complications of the disease?
If left untreated, phospholipid syndrome can lead to extremely dangerous complications. Against the background of the disease, blood clots form in the vessels, which in itself is dangerous. Blood clots clog blood vessels, disrupting normal blood circulation - tissues and organs do not receive enough nutrients and oxygen.
Often, against the background of an illness, patients develop a stroke and myocardial infarction. Blockage of the vessels of the extremities can lead to the development of gangrene. As mentioned above, patients have impaired functioning of the kidneys and adrenal glands. The most dangerous consequence is pulmonary embolism - this pathology develops acutely, and not in all cases the patient can be delivered to the hospital on time.
Pregnancy in patients with phospholipid syndrome
As already mentioned, phospholipid syndrome is diagnosed during pregnancy. What is the danger of the disease and what to do in such a situation?
Due to the phospholipid syndrome, blood clots 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 this leads to miscarriage. Even if the pregnancy is not interrupted, there is a risk of early placental abruption and the development of late preeclampsia, which is very dangerous for both mother and child.
Ideally, a woman should be tested at the planning stage. However, 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. To prevent thrombosis of the expectant mother, anticoagulants may be prescribed in small doses. In addition, a woman should regularly undergo examinations so that the doctor can notice the onset of placental abruption in time. Every few months, expectant mothers undergo a course of general strengthening therapy, taking preparations containing vitamins, minerals and antioxidants. With the right approach, pregnancies often end happily.
What does the treatment look like?
What to do if a person has phospholipid syndrome? Treatment in this case is complex, and it depends on the presence of certain complications in the patient. Since blood clots form against the background of the disease, the therapy is primarily aimed at thinning the blood. Schemetreatment usually includes the use of several groups of drugs:
- First of all, indirect anticoagulants and antiaggregants ("Aspirin", "Warfarin") are prescribed.
- Therapy often includes selective non-steroidal anti-inflammatory drugs such as Nimesulide or Celecoxib.
- If the disease 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 to suppress the activity of the immune system and reduce the production of dangerous antibodies.
- Immune globulin is sometimes given to pregnant women.
- Patients periodically take drugs containing B vitamins.
- For general healing, protection of blood vessels and cell membranes, antioxidant drugs are used, as well as drugs that contain a complex of polyunsaturated fatty acids (Omacor, Mexicor).
Electrophoresis procedures are useful for the patient's condition. When it comes to 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 carry out appropriate therapy until complete recovery (if possible).
Prognosis for patients
If phospholipid syndrome was diagnosedon time and the patient received the necessary assistance, the prognosis is very favorable. Unfortunately, it is impossible to get rid of the disease forever, but with the help of medicines it is possible to control its exacerbations and carry out preventive treatment of thrombosis. Dangerous are situations in which the disease is associated with thrombocytopenia and high blood pressure.
In any case, all patients with a diagnosis of "phospholipid syndrome" should be under the control of a rheumatologist. How often the analysis is repeated, how often you need to undergo examinations with other doctors, what drugs you need to take, how to monitor the state of your own body - the attending physician will tell about all this.
