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 jeopardizes the health of the mother and child.
Of course, many people seek additional information by asking questions about the causes of the disease. What symptoms should I look for? Is there an analysis for phospholipid syndrome? Can medicine offer effective treatments?
Phospholipid syndrome: what is it?
This disease was first 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 with a similar ailment are significant:
- Antibodies attack healthy endothelial cells, reducing the synthesis of growth factors and prostacyclin, which is responsible for the expansion of blood vessels. Against the background of the disease, there is a violation of platelet aggregation.
- Phospholipids are also contained in the walls of platelets themselves, which leads to increased aggregation, as well as rapid destruction.
- In the presence of antibodies, an increase in blood coagulability and a weakening of heparin activity are observed.
- The process of destruction does not bypass nerve cells.
Blood begins to coagulate in the vessels, forming blood clots that disrupt the blood flow, and therefore the functions of various organs - this is how the phospholipid syndrome develops. The causes and symptoms of this ailment are of interest to many people. After all, the sooner the disease is detected, the less 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. An ailment develops in the event of a malfunction of the immune system, which for one reason or another begins to produce antibodies to the cells of its own body. In any case, the disease must be provoked by something. To date, scientists have managed to identify several risk factors:
- Often, phospholipid syndrome 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.
- 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, Novokainamid, etc.
Naturally, it is important to find out why the patient developed the phospholipid syndrome. Diagnosis and treatment should identify and, if possible, eliminate the root cause of the disease.
Lesions of the cardiovascular system with phospholipid syndrome
Blood and blood vessels are the first “targets” that affect the phospholipid syndrome. Symptoms of it depend on the stage of development of the disease. Blood clots, as a rule, first form in the small vessels of the limbs. 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. Long-term malnutrition leads to necrosis and subsequent gangrene.
Thrombosis of deep veins of the extremities is also possible, which is accompanied by the appearance of edema, pain, and impaired mobility. Phospholipid syndrome can be complicated by thrombophlebitis (inflammation of the vascular walls), which is accompanied by fever, chills, redness of the skin in the affected area and acute, 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);
- superior vena cava syndrome (this condition is characterized by swelling, blueness of the skin, bleeding from the nose, trachea and esophagus);
- lower vena cava syndrome (accompanied by circulatory disorders in the lower body, swelling of the limbs, pain in the legs, buttocks, abdominal cavity and groin).
Thrombosis 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 the prolonged development of phospholipid syndrome, a 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 blood clot 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 development of a necrotic process is possible. The dangerous effects of phospholipid syndrome include renal microangiopathy, in which small blood clots form directly in the renal glomeruli. Such a 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 likelihood of developing various mental disorders.
In some patients, blood clots are found in the vessels providing blood supply to the visual analyzer. Long-term deficiency of oxygen and nutrients leads to atrophy of the optic nerve. Possible retinal vascular thrombosis followed by hemorrhage. Unfortunately, some of the eye pathologies are irreversible: visual disturbances remain with the patient for life.
Bones can also be involved in the pathological process. In humans, reversible osteoporosis is often diagnosed, which is accompanied by skeletal deformity and frequent fractures. More dangerous is aseptic necrosis of the bone.
For the disease, skin lesions are also characteristic. Often, spider veins form on the skin of the upper and lower extremities. Sometimes you can notice a very characteristic rash that resembles small, punctate hemorrhages. Some patients develop erythema on the soles of the feet and hands. There is a frequent formation of subcutaneous hematomas (for no apparent reason) and hemorrhage under the nail plate. A prolonged violation of tissue trophism entails the appearance of ulcers that heal for a long time and are difficult to treat.
We found out what constitutes phospholipid syndrome. The causes and symptoms of the disease are extremely important issues. 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. The doctor may suspect a phospholipid syndrome even during the collection of anamnesis. This thought can be prompted by the patient's presence of thrombosis and trophic ulcers, frequent miscarriages, and signs of anemia. Of course, further examinations are carried out in the future.
The analysis for phospholipid syndrome consists in determining the level of antibodies to phospholipids in the blood of patients. In a general blood test, you can notice a decrease in platelet levels, an increase in ESR, an increase in the number of white blood cells. Often, the syndrome is accompanied by hemolytic anemia, which can also be seen during laboratory studies.
Additionally, a biochemical blood test is performed. In patients, an increase in the number of gamma globulins is observed. If the liver was damaged against the background of pathology, then the amount of bilirubin and alkaline phosphatase in the blood rises. In the presence of renal disease, an increase in creatinine and urea levels can be observed.
Specific immunological blood tests are recommended for some patients. For example, laboratory tests can be performed to determine the rheumatoid factor and lupus coagulant. With phospholipid syndrome in the blood, you can detect the presence of antibodies to red blood cells, an increase in the level of lymphocytes. If there are suspicions of the presence 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?
If untreated, phospholipid syndrome can lead to extremely dangerous complications. Against the background of the disease, blood clots form in the 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 an ailment, patients develop stroke and myocardial infarction. Blockage of the vessels of the limbs can lead to the development of gangrene. As mentioned above, patients have abnormalities in the 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 a similar 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 a miscarriage. Even if the pregnancy did not end, there is a risk of early detachment of the placenta and the development of late gestosis, which is very dangerous for both the mother and the baby.
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. For the prevention of thrombosis of the expectant mother, anticoagulants in small doses can be prescribed. In addition, a woman should undergo regular examinations so that the doctor can notice in time the onset of placental abruption. Every few months, expectant mothers undergo a course of general strengthening therapy, taking medications containing vitamins, minerals and antioxidants. With the right approach, pregnancy often ends happily.
What does the treatment look like?
What to do if a person has phospholipid syndrome? The 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 diluting the blood. The treatment regimen, as a rule, includes the use of several groups of drugs:
- Indirect anticoagulants and antiplatelet agents (Aspirin, Warfarin) are primarily prescribed.
- 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 immunoglobulin intravenously.
- Patients periodically take preparations 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 is beneficial for the patient. 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 of these pathologies. It is also important to detect infectious diseases on time and conduct appropriate therapy until complete recovery (if possible).
Predictions for Patients
If the phospholipid syndrome was diagnosed on time and the patient received the necessary help, the prognosis is very favorable. Unfortunately, it is impossible to get rid of the disease forever, but with the help of medications you can control its exacerbations and carry out preventive treatment of thrombosis. Situations are considered dangerous in which the disease is associated with thrombocytopenia and high blood pressure.
In any case, all patients diagnosed with a phospholipid syndrome should be under the control of a rheumatologist. How much the analysis is repeated, how often it is necessary to undergo examinations with other doctors, what drugs should be taken, how to monitor the state of your own body - the attending physician will tell you all about this.