Ant-phospholipid syndrome (APS)
Recurrent arterial +/or VTE with no signs connective tissue disease
Antiphospholipid antibodies APL (IgM and IgG)
- Present in 2% of people (50% on those with SLE = "secondary APS")
- Generation may have trigger eg. phenytoin, chlorpromazine, quinidine and some antibiotics
- Miscellaneous triggers include: malignancy, HIV, viral infections (these usually not at VTE risk)
Clinical presentation
- VTE/PE (+/- ↑ Pulmonary BP)
- CVA or venous thromboses
- Thrombocytopenia or haemolytic anaemia
- Skin iscahemia (cyanosis/gangrene, livedo reticularis, discoid rash and chronic ulcers)
- Cardiac (valvular Libman-Sacks as in SLE) lesions
- Ocular thrombosis
- Obstetric: miscarriage, IUGR, eclampsia
- Rarely - "catastrophic APS", "Asherson syndrome" - fulminant multi organ failure
Differential
- DIC
- Endocarditis
- Thrombotic or heparin-induced thrombocytopenia
- Hypercoagulable states.
Treatment
- Anticoagualtion (INR 2-3)
- Consider Hydroxychloroquine
- Aspirin (evidence for/against is weak)
- Clopidogrel (no strong edidence)
