Antiphospholipid syndrome (APS) is an autoimmune pathology. It is based on the formation of antibodies to phospholipids, which are the main components of cell membranes. Such a disease manifests itself with problems with blood vessels, heart, other organs, as well as with pregnancy.
In the article, we consider the causes, symptoms, diagnostic methods for antiphospholipid syndrome. Clinical recommendations for the treatment of APS have passed public examination and were approved in December 2013. The recommendations also provide a detailed description of what constitutes antiphospholipid syndrome. The information provided in this document is a practical guide for medical staff working with patients diagnosed with APS.
General information
APS can be called a symptom complex, including recurrent arterial and venous thrombosis, as well as obstetric pathology. There are two types of disease:
- Primary antiphospholipid syndrome.
- Secondary AFS.
The primary one is diagnosed when the patient has not manifested other diseases besides AFS for 5 years.
Secondary is a pathology that developed against the background of another pathology (lupus erythematosus, scleroderma, rheumatoid arthritis and others).
In the first embodiment, the patient does not have erythema on the face, skin rashes, stomatitis, inflammation of the peritoneum, Raynaud's syndrome, and the blood test does not have antinuclear factor, antibodies to native DNA, and antibodies to Sm antigen.
Diagnosis of the disease
Antiphospholipid syndrome is diagnosed when people have at least one clinical and laboratory criterion for its manifestation. If there are only clinical criteria, and laboratory indicators are not available, then the diagnosis of this disease is not made. Also, do not diagnose APS in the presence of only laboratory criteria. A diagnosis of APS is excluded if a person has antiphospholipid antibodies in the blood for more than five consecutive years, but no clinical symptoms are observed.
Diagnosis of antiphospholipid syndrome has its own nuances.
Considering the fact that to determine the laboratory parameters of APS, it is required to examine the concentration of antiphospholipid antibodies in the blood at least twice, it is impossible to make an accurate diagnosis in a single examination. Only when the appropriate tests are passed twice will laboratory criteria be evaluated.
A positive test result will be considered only if the number of antibodies to phospholipids in the analysis of blood for antiphospholipid syndrome is increased twice in a row. In the event that all the time antiphospholipid antibodies were observed in an increased amount, and when re-examined were normal, this is considered a negative criterion and does not serve as a sign of this disease. Diagnosis of antiphospholipid syndrome should be carried out by a highly qualified specialist.
The fact is that a temporary increase in antiphospholipid antibodies in the blood can occur very often. In fact, it is fixed after each infectious disease, even against the background of banal otolaryngological ailments. Such a temporary increase in the level of antibodies does not require treatment and passes on its own within just a few weeks.
When the diagnosis of this syndrome is confirmed or refuted, it should not be immediately considered final, since their level can fluctuate depending on a variety of reasons, for example, such as a recent cold or stress.
Differentiation with other diseases
Antiphospholipid syndrome according to ICD 10 has a code of D 68.6. The tenth revision was held in 1989 in Geneva. His innovation was the use in the codes of diseases and numbers, and letters. Prior to this, antiphospholipid syndrome according to ICD 9 had a code of 289.81 in the class "Diseases of the blood and blood-forming organs." APS can often be confused with other pathologies. Thus, the disease must be able to distinguish from the following diseases that have similar clinical symptoms to it:
- The patient has acquired or genetic thrombophilia.
- The presence of defects in fibrinolysis.
- The development of malignant tumors of absolutely any localization.
- The presence of atherosclerosis or embolism.
- The development of myocardial infarction with cardiac ventricular thrombosis.
- The development of decompression sickness.
- The patient has thrombotic thrombocytopenic purpura or hemolytic uremic syndrome.
What tests should be taken to diagnose APS
As part of the diagnosis of a disease such as antiphospholipid syndrome, it is necessary to donate blood from a vein. This is done on an empty stomach in the morning. Moreover, a person should not be cold.
In the event that the patient is not feeling well, it is impossible to take an analysis for antiphospholipid syndrome. It is required to wait for the full normalization of the condition, and only then do the necessary tests.
Immediately before passing such tests, there is no need to follow any special diet, but it is very important to limit alcohol, smoking and eating junk food. Analyzes can be taken absolutely on any day of menstruation, if it concerns a woman. So, in the framework of the diagnosis of antiphospholipid syndrome, the following studies should be performed:
- Antibodies to phospholipids such as "IgG" and "IgM".
- Antibodies to cardiolipin type "IgG" and "IgM".
- Antibodies to glycoprotein type "IgG" and "IgM".
- Study of lupus anticoagulant. It is considered optimal to determine this parameter in the laboratory according to the Russell test using viper venom.
- Complete blood count with platelet count.
- Performing a coagulogram.
The indicated analyzes are quite sufficient for making or for refuting the corresponding diagnosis. On the recommendation of a doctor, you can take other additional tests for indicators that characterize the state of the blood coagulation system. For example, you can additionally pass D-dimer, thromboelastogram, and so on. However, such additional tests will not allow to clarify the diagnosis, but on their basis it will be possible to most accurately assess the risk of thrombosis and the coagulation system as a whole.
Antiphospholipid Syndrome and Pregnancy
In women, AFS can cause miscarriage (if the period is short) or premature birth.
An ailment can lead to a delay in the development or intrauterine death of the fetus. Abortion occurs most often in the 2nd and 3rd trimesters. If there is no therapy, then such a sad outcome will be in 90–95% of patients. With timely proper treatment, an unfavorable development of pregnancy is likely in 30% of cases.
Options for pregnancy pathology:
- The death of a healthy fetus for no apparent reason.
- Preeclampsia, eclampsia, or placental insufficiency for up to 34 weeks.
- Spontaneous abortions of up to 10 weeks, with no chromosomal abnormalities in the parents, as well as hormonal or anatomical abnormalities of the genital organs in the mother.
With antiphospholipid syndrome, pregnancy can proceed normally.
Clinical manifestations may be absent. In such cases, the disease is detected only when laboratory tests are performed. Acetylsalicylic acid up to 100 mg per day is prescribed as a treatment, however, the benefit of such therapy has not been conclusively established.
Asymptomatic antiphospholipid syndrome is treated with "Hydroxychloroquine." Especially often it is prescribed for concomitant diseases of connective tissue, such as systemic lupus erythematosus. If there is a risk of thrombosis, Heparin is prescribed in a prophylactic dose.
Next, we find out how the treatment of this pathology is currently being carried out.
Treatment
Currently, unfortunately, the treatment of antiphospholipid syndrome is a very difficult task, since today there are still no accurate and reliable data on the mechanism and reason for the development of this pathology.
Therapy is currently aimed at eliminating and preventing thrombosis. Thus, the treatment is inherently symptomatic and does not allow to achieve an absolute cure for this disease. This means that such therapy is carried out for life, since it makes it possible to minimize the risks of thrombosis, but at the same time does not eliminate the disease. That is, it turns out that according to the state of medicine and the knowledge of science, today patients should eliminate the symptoms of APS for life. In the treatment of this disease, two main directions are distinguished, which are the relief of frolicing thrombosis, as well as the prevention of repeated episodes of thrombosis.
Emergency treatment
Against the background of the catastrophic antiphospholipid syndrome, urgent treatment is carried out for patients, which is carried out in the conditions of resuscitation. To do this, use all available methods of anti-inflammatory and intensive care, for example:
- Conducting antibiotic therapy, which eliminates the foci of infection.
- The use of "Heparin." In addition, low molecular weight drugs such as Fraxiparin along with Fragmin and Kleksan are used. These medicines help reduce blood clots.
- Treatment with glucocorticoids in the form of "Prednisolone", "Dexamethasone" and so on. These medicines allow you to stop systemic inflammatory processes.
- The simultaneous use of glucocorticoids with Cyclophosphamide for the relief of severe systemic inflammatory processes.
- Intravenous administration of immunoglobulin against thrombocytopenia. Such a measure is advisable in the presence of a low number of platelets in the blood.
- If there is no effect from the use of glucocorticoids, immunoglobulin and Heparin, experimental genetic engineering medicines like Rituximab and Eculizumab are introduced.
- Plasmapheresis is carried out only with an extremely high titer of antiphospholipid antibodies.
Some studies have proven the effectiveness of Fibrinolysin along with Urokinase, Alteplase and Antistreplaza in the relief of a catastrophic form of the syndrome. But I must say that these drugs are not prescribed continuously, since their use is associated with high risks of bleeding.
Drug treatment of thrombosis
As part of the prevention of thrombosis, people suffering from this syndrome need to use drugs for life that reduce blood coagulation. The choice of drugs directly determines the clinical features of this disease. Today, doctors are advised to adhere to the following tactics during the prevention of thrombosis in patients suffering from antiphospholipid syndrome:
- With AFS with antibodies to phospholipids, against the background of which there are no clinical episodes of thrombosis, you can limit yourself to low doses of acetylsalicylic acid at 75 milligrams per day. “Aspirin” in this case is taken for life or until the treatment tactics change. In the event that this syndrome is secondary (for example, proceeds against a background of lupus erythematosus), patients are recommended to use “Hydroxychloroquine” simultaneously with “Aspirin”.
- In case of APS with the presence of venous thrombosis episodes, it is recommended to use Warfarin. In addition to Warfarin, Hydroxychloroquine may be prescribed.
- In case of antiphospholipid antibody syndrome with the presence of episodes of arterial thrombosis, Warfarin and Hydroxychloroquine are also recommended. And in addition to Warfarin and Hydroxychloroquine, in case of a high risk of thrombosis, Aspirin is also prescribed in a low dosage.
Complementary Medications for Treatment
In addition to any of the above treatment regimens, certain drugs may be prescribed in order to correct existing violations. For example, against the background of moderate thrombocytopenia, low dosages of glucocorticoids are used - Metipred, Dexamethasone, Prednisolone, and so on. In the presence of clinically significant thrombocytopenia glucocorticoids, as well as Rituximab are used. Immunoglobulin may also be used.
In the event that the treatment does not allow to increase platelets in the blood, then surgical removal of the spleen is performed. In the presence of kidney pathologies against the background of this syndrome, drugs from the category of inhibitors are used, for example, “Captopril” or “Lisinopril”.
New drugs
Recently, the development of new drugs that prevent thrombosis, which include heparinoids, and in addition, receptor inhibitors, such as Ticlopidine along with Tagren, Clopidogrel, and Plavix, have been actively carried out recently.
According to preliminary information, it is reported that these drugs are very effective in antiphospholipid syndrome. It is likely that they will soon be introduced into the treatment standards recommended by international communities. To date, these drugs are used as part of the treatment of antiphospholipid syndrome, but each doctor tries to prescribe them according to his own scheme.
If there is a need for surgery for this syndrome, you should take anticoagulants in the form of "Warfarin" and "Heparin" as long as possible. You need to cancel them for the minimum possible period of time before the operation. Resume taking "Warfarin" is necessary after surgery.
In addition, people who suffer from antiphospholipid syndrome need to get out of bed and start moving as soon as possible after surgery. It will not be superfluous to wear stockings from special compression knitwear, which will allow for additional prevention of the risk of thrombosis. Instead of special compression underwear, a simple wrapping of the legs with elastic bandages is suitable.
Clinical recommendations for antiphospholipid syndrome should be strictly observed.
What other drugs are used
In the treatment of this disease, drugs from the following groups are used:
- Treatment with antiplatelet agents and indirect anticoagulants. In this case (in addition to Aspirin and Warfarin), Pentoxifylline is often used.
- The use of glucocorticoids. In this case, the drug "Prednisolone" can be used. In this case, its combination with immunosuppressants in the form of Cyclophosphamide and Azathioprine is also possible.
- The use of aminoquinoline preparations, for example, Delagil or Plaquenil.
- The use of selective non-steroidal anti-inflammatory drugs in the form of Nimesulide, Meloxicam or Celecoxib.
- As part of obstetric pathologies, Immunoglobulin is used intravenously.
- Treatment with B vitamins.
- The use of polyunsaturated fatty acid preparations, for example, Omacor.
- The use of antioxidants, such as Mexico.
Widespread use has not yet been received, but the drugs in the following categories are very promising in the treatment of antiphospholipid syndrome:
- The use of monoclonal antibodies to platelets.
- Treatment with anticoagulant peptides.
- Use of apoptosis inhibitors.
- The use of systemic enzyme therapy drugs, for example, "Wobenzym" or "Flogenzima."
- Treatment with cytokines (mainly Interleukin-3 is used today).
In the framework of preventing re-thrombosis, mainly indirect anticoagulants are used. In cases of the secondary nature of antiphospholipid syndrome, treatment is carried out against the background of adequate therapy for the underlying disease.
The prognosis against the background of pathology
The prognosis for this diagnosis is ambiguous and primarily depends on the timeliness of treatment, as well as on the adequacy of therapeutic methods. No less important is the patient's discipline, compliance with all the necessary instructions of the attending physician.
What other recommendations are there for antiphospholipid syndrome? Doctors advise not to conduct treatment at their own discretion or on the advice of "experienced", but only under the supervision of a doctor. Remember, the selection of medicines for each patient is individual. Drugs that have helped one patient can greatly aggravate the situation of another. Doctors also advise people with APS to regularly monitor their laboratory findings. This is especially true for pregnant women and for those women who plan to become mothers.
Which doctor should I contact?
The treatment of such a disease is carried out by a rheumatologist. , , -. , , , , , , , , , , .