Antiphospholipid syn

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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:

• recurrent thromboembolic events
• recurrent fetal loss
• livedo reticularis
• high titre sustained IgG anticardiolipin antibody or lupus anticoagulant with abnormal PTT. (NB a number of infections can induce anti-phospholipid antibodies eg syphilis, Lyme disease, HIV-1)

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

• thrombophlebitis
• pulmonary emboli
• IVC occlusion
• Budd-Chiari
• sagittal vein and cavernous sinus thrombosis

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

©Charles Gomersall, August, 2008 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
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