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Antiphospholipid syndrome - Classification
Last reviewed: 07.07.2025
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There are primary and secondary antiphospholipid syndrome. The development of secondary antiphospholipid syndrome is associated with autoimmune, oncological, infectious diseases, as well as with the effects of certain drugs and toxic substances.
Primary antiphospholipid syndrome can be considered in the absence of the diseases and conditions listed above.
A number of authors distinguish the so-called catastrophic antiphospholipid syndrome, characterized by sudden and rapidly developing multiple organ failure, most often in response to provoking factors (infectious diseases or surgical interventions). Catastrophic antiphospholipid syndrome is characterized by: acute respiratory distress syndrome, cerebrovascular and coronary circulatory disorders; stupor, disorientation; possible development of acute renal and adrenal insufficiency, thrombosis of large vascular trunks. Without timely treatment, mortality reaches 60%.
[ Classification of antiphospholipid syndrome (Alekberova Z.S., Nasonov E.L., Reshetnyak T.M., 2000)
Clinical variants
- Primary antiphospholipid syndrome.
- Secondary antiphospholipid syndrome in:
- rheumatic and autoimmune diseases;
- malignant neoplasms;
- use of medications;
- infectious diseases;
- in the presence of other reasons.
- Other options:
- catastrophic antiphospholipid syndrome;
- a number of microangiopathic syndromes (thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, HELLP syndrome);
- hypothrombinemia syndrome;
- disseminated intravascular coagulation;
- antiphospholipid syndrome in combination with vasculitis.
Serological variants of antiphospholipid syndrome
- Seropositive antiphospholipid syndrome with the presence of anticardiolipin antibodies and/or lupus anticoagulant.
- Seronegative antiphospholipid syndrome:
- with the presence of antiphospholipid antibodies that react with phosphatidylcholine;
- with the presence of antiphospholipid antibodies that react with phosphatidylethanolamine; ✧ with the presence of (32-glycoprotein-1-cofactor-dependent antiphospholipid antibodies.