Antiphospholipid syndrome
This is actually down in the textbooks as an acquired coagulation abnormality rather than a rheumatology element but lets chat about it!
So called APS is an autoimmune condition characterised by recurrent thrombosis, recurrent pregnancy loss and other obstetric complications, and the presence of autoantibodies directed against phospholipids. Thrombocytopenia is also common in these patients.
It is thought that the prothrombotic effect that we see in APS is due to the effects that antiphospholipid antibodies have on beta2-GPI, or through inhibition of protein C, and therefore initiating thrombosis.
APS patients can have bleeding tendencies (due to the thrombocytopenia), they can also present with rashes, chronic headaches, dementia and seizures.
Most cases occur with no family history, as the condition is acquired (autoimmune) rather than genetic.
Diagnosis is based on clinical and biochemical findings:
- Clinical criteria
- ≥1 episode of vascular thrombosis
- Obstetric morbidity - ≥3 consecutive spontaneous miscarriages or ≥1 unexplained foetal death
- Lab criteria - presence of any of the following on two occasions at least 12 weeks apart
- Lupus anticoagulant
- Anticardiolipin antibodies
- Anti-b2 glycoprotein I antibodies
Treatment is lifelong anticoagulation therapy (warfarin), aspirin and LMWH may be used during pregnancy.