What Is Antiphospholipid Antibody Syndrome?

Antiphospholipid antibody syndrome, also known as Hoghes syndrome, is a clinical syndrome characterized by recurrent thrombotic events and miscarriage, and the presence of antiphospholipid antibodies (APL) in serum. The clinical manifestations are mainly thrombosis. Small and medium blood vessels are the most common. Thrombosis can be formed in both arteries and veins, and venous thrombosis is more common.

Antiphospholipid antibody syndrome, also known as Hoghes syndrome, is a clinical syndrome characterized by recurrent thrombotic events and miscarriage, and the presence of antiphospholipid antibodies (APL) in serum. The clinical manifestations are mainly thrombosis. Small and medium blood vessels are the most common. Thrombosis can be formed in both arteries and veins, and venous thrombosis is more common.

English name

antiphospholipid-antibody syndrome

Visiting department

Hematology

Disease site

blood

Common symptoms

Thrombosis symptoms, habitual abortion, etc.

Causes and common diseases of antiphospholipid antibody syndrome

It can be clinically divided into primary APS (PAPS or 1APS), whose clinical manifestations cannot meet the diagnostic criteria for other diseases, and secondary APS (SAPS or 2APS). The latter can be secondary to systemic lupus erythematosus and rheumatoid joints. Connective tissue diseases such as inflammation, systemic sclerosis and Sjogren's syndrome. Another rare clinical type, called malignant APS (catastrophic APS), is characterized by the progressive appearance of massive thrombosis in a short period of time (days to weeks), affecting the central nervous system, kidneys, lungs and heart Important organs, causing organ failure and death.

Anti-phospholipid antibodies refer to lupus anti-coagulant (LA), anti-cardiolipid antibody (ACL) or a group of autoantibodies against other phospholipids or phospholipid complexes. The most basic pathological feature of antiphospholipid antibody syndrome is that all clinical manifestations of thrombosis are related to it. It was thought that anti-phospholipid antibodies were only directed against negatively charged phospholipids. Now it is found that anti-phospholipid antibodies may act more directly on one or more plasma proteins bound to phospholipids or the complexes of these proteins and phospholipids, the most important of which is 2. Glycoprotein I (2-glycoprotein, 2-GP) and prothrombin.

Differential diagnosis of antiphospholipid antibody syndrome

Autoimmune diseases characterized by clinical symptoms of various thrombotic symptoms, habitual abortion, thrombocytopenia, and anti-phospholipid antibodies such as myocardial phospholipid antibody and lupus anticoagulant factor. In most cases, APS and systemic lupus erythematosus (SLE ) Coexistence, a few can occur glomerular lesions.

Thrombosis

Thrombosis is the main clinical manifestation of antiphospholipid antibody syndrome. Thrombosis can occur in blood vessels in any part of the body. Peripheral blood vessels, cerebral blood vessels, and blood vessels of the heart, lungs, kidneys and other organs are often involved. Thrombus is usually single. The occurrence of thrombus has no obvious relationship with the change of serum antiphospholipid antibody titer, but sometimes the formation of large thrombus is often accompanied by a decrease in antibody titer.

2. Performance of pregnancy

About half of pregnant women with antiphospholipid antibody syndrome can have miscarriages. Miscarriages generally occur in the second and third trimesters of pregnancy, mainly due to placental vascular thrombosis and placental infarction that lead to decreased placental function. The occurrence of abortion is closely related to IgG anti-phospholipid antibodies. The incidence of abortion is extremely low in IgM and IgA anti-phospholipid antibody-positive patients. Other manifestations of antiphospholipid antibody syndrome during pregnancy are: preeclampsia, pregnancy-induced hypertension, delayed fetal growth and development, fetal distress and premature birth, etc., their incidence is about 30%. These clinical manifestations are likely to aggravate a small number of pregnant women with non-antiphospholipid antibody syndrome during pregnancy. High titers of IgG antiphospholipid antibodies may increase the risk of miscarriage, but it is unclear whether it causes other gynecological complications. However, most pregnant women with non-antiphospholipid antibody syndrome, although the serum antiphospholipid antibody is positive, have no significant increase in clinical complications, and can be pregnant normally.

3. Thrombocytopenia

Patients with phospholipid antibody syndrome often show moderate thrombocytopenia (typically> 50 × 10 / L). Although antiphospholipid antibody syndrome has thrombocytopenia, it is still prone to thrombosis and rarely has bleeding symptoms. Thrombocytopenia common in systemic lupus erythematosus is associated with the presence of antiphospholipid antibodies. Thrombocytopenia occurs in about 40% of patients with systemic lupus erythematosus positive for antiphospholipid antibodies, but only 10% of systemic lupus erythematosus patients with negative antiphospholipid antibodies have thrombocytopenia. Conversely, 70% to 80% of patients with systemic lupus erythematosus with thrombocytopenia are positive for antiphospholipid antibodies.

4. Children's antiphospholipid syndrome

Children's antiphospholipid syndrome is more common in women (male to female ratio is about 2: 3). The age of onset is 8 months to 16 years (mean 10 years). Most children are prone to recurrent thrombosis. The ratio of arteriovenous thrombosis was 1: 0.6, and about 14% of patients had arteriovenous thrombosis at the same time. But younger children are more likely to develop arterial thrombosis than older ones, and cerebral arterial thrombosis is more common. Pulmonary embolism is less common in children than in adults.

5. Neuropsychiatric injury

In SLE and other autoimmune diseases, 38% of LA positives and 49% of ACA positives have neuropsychiatric symptoms. In contrast, 21% of LA negative patients and 12% of ACA antibody negative patients. The main manifestations of neuropsychiatric symptoms are cerebrovascular accidents, including cerebral thrombosis, cerebral hemorrhage, mental and behavioral abnormalities, epilepsy, chorea, and spinal cord disease. Most of these disorders are caused by focal cerebral ischemia or cerebral thrombosis. The neuropsychological system damage is mainly caused by APA by damaging vascular endothelial cells, activating platelets, and affecting the blood coagulation system to cause thrombosis. It may also be that APA directly cross-reacts with brain phospholipids and causes diffuse damage to brain tissue.

Antiphospholipid antibody syndrome test

Anti-phospholipid antibodies, anti-cardiolipin antibodies APL antibodies such as LA, ACL antibodies are positive, and their clinical significance is greater when their titers are high. Several international standardization seminars have developed a unified ELISA method for the detection of APL, which can be used for quantitative or semi-quantitative determination of IgG, IgA, and IgM of APL. It is now recommended to express the results of APL experiments with negative, low, moderate, and high positives, in order to improve the consistency and repeatability of test results in various laboratories.

CT scans are generally not found abnormally, but magnetic resonance imaging examination can find a small area of increased T1 and T2 signals.

Antiphospholipid antibody syndrome treatment principles

The treatment of APS mainly includes anticoagulation and antiplatelet therapy, such as aspirin, ticlopidine, heparin, and warfarin. Adrenocortical hormones are limited to the treatment of blood system diseases such as thrombocytopenia and autoimmune hemolytic anemia caused by APS. , And critically ill APS and APS-induced transmyelopathy.

Antithrombotic therapy: In the acute phase, heparin can be used to block thrombosis. For patients with arteriovenous thrombosis, anticoagulants can be taken orally. For those who have used sufficient warfarin for anticoagulation and who have repeated thrombosis, they can subcutaneously inject sufficient heparin, or use immunosuppressants (cyclophosphamide), hormones, heparin and warfarin for anticoagulation. Combination therapy.