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Antiphospholipid syndrome: classification
Medical expert of the article
Last reviewed: 23.04.2024
Isolate the primary and secondary antiphospholipid syndrome. The development of secondary antiphospholipid syndrome is associated with autoimmune, oncological, infectious diseases, as well as with the impact of certain drugs and toxic substances.
The primary antiphospholipid syndrome can be said 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). For a catastrophic antiphospholipid syndrome are characterized by: acute respiratory distress syndrome, impaired cerebral and coronary circulation; stupor, disorientation; possible development of acute renal and adrenal insufficiency, thrombosis of major vascular pathways. Without timely treatment, the mortality rate reaches 60%.
[Classification of antiphospholipid syndrome (Alekberova ZS, Nasonov EL, Reshetnyak TM, 2000)
Clinical Options
- Primary antiphospholipid syndrome.
- Secondary antiphospholipid syndrome with:
- rheumatic and autoimmune diseases;
- malignant neoplasms;
- use of medicines;
- infectious diseases;
- presence of other reasons.
- Other options:
- catastrophic antiphospholipid syndrome;
- a number of microangiopathic syndromes (thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, HELLP- syndrome);
- syndrome of hypothrombinemia;
- 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 reacting with phosphatidylcholine;
- with the presence of antiphospholipid antibodies reacting with phosphatidylethanolamine; ✧ with the presence of (32-glycoprotein-1-cofactor-dependent antiphospholipid antibodies.