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Anti-phospholipid
syndrome
Used to be considered to be part of the clinical spectrum of SLE but while
the 2 syndromes often co-exist, SLE is not necessary for the diagnosis of
antiphospholipid syndrome
Definition
There is as yet no agreed definition of APS but the consensus opinion seems
to be that the following are required, although they need not be present
simultaneously:
Clinical syndromes
Arterial occlusion syndromes
- arterial occlusion occurs as discrete episodes
- usually occurs in single rather than multiple vessels
- can affect any size of vessel
- in individual patients the size of vessel and the body area affected is
usually consistent from episode to episode eg patients who have had a stroke
tend to have recurrent strokes
- no recognised triggers
Brain
- stroke. Accounts for 36% of new strokes in patients < 50 years.
- recurrence rate almost 20% per year
- small vessel occlusion leading to confusion
Kidney
- modest proteinuria most common clinical manifestation
- hypertension
- haematuria and hypocomplementaemia not features
- renal insufficiency
Catastrophic occlusion syndrome
- rare
- multiple widespread infarctions over days to weeks
- may be precipitated by viral infection or allergic reaction
- leukocytosis
- high levels of tissue enzymes
- intravascular coagulation
- resembles sepsis, DIC or TTP
- differentiation from the above difficult but consumption of coagulation
factors, fragmented RBCs and biopsy evidence of microvascular occlusion,
hallmarks of the above, are usually absent
- frequently lethal
Venous occlusion syndromes
Valvular heart disease
- patients with long standing APS sometimes develop Libman Sacks type
vegetations
- patients with stroke and vegetations rarely have evidence of emboli from
cardiac valves or cardiac thrombus
Treatment
- No good controlled data on which to base treatment.
- In general treatment consists of either anti-coagulation with heparin and
then warfarin or anti-platelet medication in the form of aspirin or both
Virtually all surviving patients with catastrophic vascular occlusion
syndrome have received emergency treatment with high-dose corticosteroid,
anti-coagulation, cyclophsophamide +/- plasmaphoresis and IV immunoglobulin
© Charles Gomersall December 1999 |
