APLA are associated with a number of disease states (Table 19.2). The best described conditions are venous thrombosis, arterial thrombosis, neurological disease, frequent miscarriages, and thrombocytopenia.
Venous thrombosis. Venous thrombosis was the first described manifestation of APLA and is the one most clinically predominant. Overall, retrospective studies show that 31% of patient with APLA have venous thrombosis. Patients with lupus and APLA have a thrombosis rate of 42%; patients with infectious or drug-induced APLA have a thrombosis rate of less than five percent. Patients with APLA are over-represented in young patients with deep vein thrombosis. Prospective studies have demonstrated a relative risk for venous thrombosis of 5.3 for patients with IgG anticardiolipin antibodies. Patients with APLA-associated venous thrombosis may be difficult to treat. These patients have high rates of recurrent thrombosis (20-50%/ year) if anticoagulation is stopped. Occasional patients may be refractory to warfarin and will need to be on long-term heparin therapy.
Arterial disease. The incidence of APLA is increased in young patients with myocardial infarction and especially stroke. APLA are also found in a higher proportion in patients with peripheral vascular disease and may be predictive graft failure. Prospective studies have demonstrated that patients with APLA have higher rates of saphenous bypass vein occlusion and re-occlusion of PTCA.
Neurological disease. A variety of neurological disorders have been associated with APLA. The underlying cause of these disorders appears to be thrombosis. Some patients have large vessel disease while more often patients have small vessel involvement. Patients with APLA often will have multiple MRI abnormalities consistent with small white matter infarcts. The neurological diseases include:
Stroke. APLA is found in 10-46% of young patients with stroke and in 10% of stroke patients overall. Stroke patients with APLA tend to be younger (42 years vs 62 years). These patients also have a recurrence rate of 6-30%/year and a mortality rate of 10%/year. Certain groups of patients appear to be at even higher risk of recurrence. These would include SLE patients with APLA and patients with Sneddon's syndrome (described below).
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