An autoimmune disorder of recurrent thrombosis and/or pregnancy loss. Can exist in isolation, else in association with systemic lupus erythematosus. In children, development of SLE appears to be inevitable.
A mixed bag of conditions, despite the name, where there may be antiphospholipid antibodies (of one sort or another e.g. anti-plasma protein β2-glycoprotein 1), or positive anticardiolipin, or positive lupus anticoagulant tests (persistently).
- Livedo reticularis
- Neurological manifestations esp. migraine
- Haemolytic anaemia
Clearly some of these are autoimmune rather than thrombotic in nature. If antiphospholipid antibodies are found incidentally, thrombosis is unusual. But can be catastrophic viz microangiopathy, pulmonary haemorrhage, ARDS, cerebral/adrenal infarction, Budd-Chiari syndrome.
Acute treatment is with IV or subcut heparin, followed by warfarin (target INR 2-3). Aspirin may be added if problematic. Hydroxychloroquine should probably always be used. Avoid smoking, oestrogens, cocaine etc. Plasma exchange and Rituximab may be used in life-threatening cases.
Since the risk of thrombosis is so low if antiphospholipid antibodies are found incidentally, unless there is a suggestion of lupus, prophylaxis is probably unnecessary. If in doubt or if some other risk factor, low dose aspirin seems reasonable. Potential complications of warfarin therapy must be considered.
Current Opinion in Hematology. 13(5):366-75, 2006
- Hughes Syndrome Foundation - overview of the condition
- Hughes Syndrome Foundation - making the diagnosis