Antiphospholipid syndrome (APS)

Antiphospholipid syndrome (APS)

What is it?

In some diseases (systemic lupus erythematosus [70%] cases, systemic scleroderma, rheumatoid arthritis, malignancy, chronic infections, etc.) developed antibodies capable of attacking phospholipids — components of cell membranes. Attached to the vessel walls, platelets, directly entering the blood coagulation reaction, such antibodies to phospholipids lead to the development of thrombosis.

Moreover, some researchers believe that perhaps direct "toxic" effects of this antibody on body tissues. The complex is manifested with symptoms called antiphospholipid syndrome (APS), and in 1994, at the International Symposium for antibodies to phospholipids were asked to call APS Hughes syndrome (Hughes) — on behalf of the British rheumatologist who first described it, and who has made the greatest contribution to the study of this problem.

Antibodies to phospholipids are a myriad of: antibodies to cardiolipin, lupus anticoagulant, b2-glycoprotein-1-kofaktorzavisimye antibodies, antibodies to coagulation factors, antibodies to the substance, on the contrary, hindering the process and many, many more. In practice most often determined by the first two — cardiolipin antibodies, lupus anticoagulant.

What are the symptoms?

The clinical picture in antiphospholipid syndrome can be very different and will depend on:

  • the size of the affected vessels (small, medium, large);
  • speed vessel closure (slow closing its lumen thrombus which it grew, or fast — Airborne thrombus, which "migrated" into this vessel from the other);
  • their function (artery or vein);
  • coarse (the brain, lungs, heart, skin, kidney, liver).

If thrombosing small vessels, this leads to a relatively mild organ dysfunction. So, with occlusion of small branches of the coronary arteries to the heart is impaired ability of individual sections of the heart muscle to contract, while the closure of the lumen of the main trunk coronary artery causes myocardial infarction.

Thrombosis Symptoms usually appear quickly, gradually, organ dysfunction increases gradually, simulating any chronic disease (Cirrhosis, Alzheimer's disease). Thrombotic occlusion of the vessel to break away, on the contrary, lead to the development of "catastrophic breaches" of organ functions. Thus, pulmonary thromboembolism seen episodes of breathlessness, chest pain, cough, in most cases it leads to death.

Antiphospholipid syndrome can mimic a variety of diseases, but some of the symptoms you should pay special attention.

Quite often, in antiphospholipid syndrome are livedo reticularis (lacy, thin mesh of vessels on the surface of the skin, which is best seen in the cold), chronic leg ulcers that are difficult to treat, peripheral gangrene (necrosis of the skin or even individual fingers or toes).

Men more often than women, a manifestation of antiphospholipid syndrome may be a myocardial infarction.

The women — is often ischemic (stroke, especially up to 40 years, headaches, migraine-like).

Vascular lesions of the liver may lead to an increase in its size, ascites (accumulation of fluid in the abdominal cavity), increasing the concentration of liver enzymes (aspartate and alaninaminotransferrazy) in blood vessels affected if renal hypertension develops (in this regard require special attention to people with which the pressure, particularly the lower, high changes frequently during the day).

Thrombosis of placental arteries can occur in utero fetal death or premature birth. It is associated with antiphospholipid syndrome that female patients with systemic lupus erythematosus, can not "save" her pregnancy, which often end in miscarriage.

As suspected?

The presence of antiphospholipid syndrome may be suspected in the following situations:

  • If a person is sick with systemic lupus erythematosus (incidence of antiphospholipid syndrome in this disease is extremely high).
  • If a person under the age of 40 years found no signs of vascular thrombosis.
  • If thrombosing vessels for which it is not very common, for example, blood vessels, supplying the intestine. They lead to blockage "abdominal pectoris." This colorful name for this disease arose by analogy with angina — "angina pectoris." "Abdominal toad" is characterized by the appearance of crushing, compression of abdominal pain occurring after a heavy meal. The more people eat, the more you need the blood digestive tract to digest food. If the lumen of blood vessels narrowed blood clots, the blood supply to the abdominal organs not getting enough, they do not have enough oxygen in their metabolic products accumulate — there is pain.
  • If the blood platelet count is reduced and there is no any haematological disease.
  • If a woman has had two or more miscarriages, and gynecologists can not pinpoint the cause.
  • If I have a heart attack at the person under 40 years of age.

Treatment

First, antiphospholipid syndrome treated only under the supervision of a physician, rheumatologist.

If antiphospholipid syndrome developed against the background of autoimmune diseases (eg, systemic lupus erythematosus), you need to treat this disease, trying to reduce its activity. If can achieve this, the serum will decrease the amount of antibodies to phospholipids. The lower the content in the blood, the less chance of thrombosis. Therefore, it is important to take a sick doctor's appointments basic therapy (corticosteroids, cytotoxic agents).

At very high titer (amount of concentration) antibodies may be an issue on plasmapheresis (blood purification).

Maybe the doctor will prescribe any medication that will reduce the likelihood of thrombosis, acting directly on the blood coagulation system. For appointment need strong indications: use should significantly exceed the side effects. Contraindications to receive these drugs are pregnancy (may cause a disorder of neural development in the fetus) and ulcerative disease of the gastrointestinal tract. It should weigh the "pros" and "cons" if the patient has liver disease or kidney disease.

Anti-malarial drugs (eg, hydroxychloroquine) combine the anti-inflammatory effect with the ability to inhibit the aggregation (clumping) of platelets, which also contributes to the prevention of thrombosis.

Women with antiphospholipid syndrome should postpone pregnancy until the normalization of laboratory values. If the syndrome developed after conception, you should consider the introduction of an immunoglobulin or small doses of heparin.

Weather will largely depend on the timely initiation of treatment and discipline of the patient.

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