Antiphospholipid syndrome

Antifosfolipidový syndrom (APS) is a disorder characterized by production of non-specific autoantibodies. These antibodies cause a hypercoagulable state by several mechanisms. It is one on the most common acquired thrombophilic states.

APS is an autoimmune disease with clinical presentation consisting of closing of the arteries or/and veins in any organ or tissue (deep vein thrombosis, thrombosis of cerebral sinuses, stroke, migraine, epilepsy, thrombocytopenia, hemolytic anemia, pulmonary embolism, pulmonary hypertension, livedo reticularis, purpura, heart valve disease, amaurosis, retinal thrombosis, myocardial infarction, vascular necrosis of bones, adrenal infarction) and also pregnancy complications like spontaneous abortion mainly after 10. week of pregnancy or preeclampsia.

The laboratory criteria is the detection of at least one of these antibodies lupus antikoagulans (LA), anti-cardiolipin antibodies (aCL class IgG or IgM) or antibodies against β2-glykoprotein I (anti β2-GP I class IgG or IgM) in minimal interval of 12 weeks between individual laboratory analysis.

APS can be divided into two general forms: secundary, which is based on the presence of other underlying disease -the most commonly systemic lupuserythematodes (SLE), and primary form, with undetected cause of production of antiphospholipid antibodies.

Clinical presentation

• recurring arterial/ venous trombosis,
• spontaneus abortion in women,
• thrombocytopenia,
• epilepsy,
• transversal myelitis,
• trombocytopenic purpura.

Characteristic and diagnosis

• They can demonstrate also in frame of other systemic disease (SLE) → secundary antiphospholipid syndrome (called as anticardiolipin),
  • antibodies against various phospholipids, phospholipid-protein complexes,
  • spontaneously prolonged activated partial thromboplastin time (aPTT), although the state is not accompanied by the higher risk of bleeding, on the contrary; the risk of thrombotic complications is higher,
  • in some cases we can detect lupus anticoagulans (circulating anticoagulant factor),
  • poor function of platelets and lower number of platelets,
• primary antiphospholipid syndrome leads to reproductive disorders (multiple abortions).

Antiphospholipid antibodies

• antiphospholipid antibodies against: phosphatidylserine , phosphatidylethanolamine, phosphoric acid, phosphatidylglycerol, phosphatidyl-β2-glycoprotein (a component of cardiolipin), annexin V (important for the diagnosis of antiphospholipid syndrome),
• antibodies IgG, IgA, IgM,
• significant factor in case of unsuccessful reproduction (IgG),
• examined by ELISA test,
• phospholipid antigens are the component of all cell membranes, including cardiolipin etc.

Therapy

• Antiagregants (antiplatelet drugs),
• acetylsalicylic acid,
• heparin,
• corticosteroids.

Links

Related articles

• Autoprotilátky

References

• MUDr. Alena Buliková, prof. MUDr. Miroslav Penka, CSc.: Antifosfolipidový syndrom

Kategorie:Imunologie Kategorie:Patofyziologie Kategorie:Hematologie Kategorie:Revmatologie Kategorie:Nefrologie Kategorie:Vnitřní lékařství