In 1983 we described a complex clinical syndrome, characterised by thrombosis, recurrent abortion, neurological disease and anti-phospholipid antibodies. The livedo reticularis is often florid on the knees. This may or may not be associated with mild to moderate Raynaud's phenomenon. These patients' blood pressures often fluctuate, apparently correlating with the severity of the livedo, suggesting a possible reno-vascular aetiology. However, this group of patients rarely has primary renal disease.
Some patients have chorea.
The patients may develop transient cerebral ischaemic attacks or visual field defects, or more significantly, progressive cerebral ischaemia. Two other features of the syndrome are a tendency to multiple spontaneous abortions and peripheral thrombosis, often with multiple leg and arm vein thrombosis. We have also seen Budd-Chiari syndrome and renal vein thrombosis in some of these patients. To my mind, however, the most striking, and often the most serious feature of this disease is the tendency to thrombosis, particularly cerebral thrombosis. The finding that many of these patients may have high titres of circulating anti-cardiolipin antibodies leads us to believe that a new line of investigation may be possible in such patients.