Thrombotic microangiopathic antiphospholipid syndromes: a continuum of conditions?

The original description and definition of the antiphospholipid syndrome (APS) included predominantly thromboses of large veins and large arteries (deep vein thromboses [DVT] and strokes), fetal losses and thrombocytopenia (mild to moderate). The thromboses and fetal losses were often recurrent. Small vessel occlusions did occur (affecting renal and retinal skin vessels, etc.) but these did not dominate the clinical picture. The systemic nature of the syndrome was recognized in 1992 and is referred to as Hughes’ Syndrome. In 1992, a new subset consisting predominantly of small vessel microangiopathy, with considerably less large vessel occlusive disease, was defined as the catastrophic antiphospholipid syndrome (CAPS) and the eponym Asherson’s Syndrome has been attached. Severe thrombocytopenia and features of the systemic inflammatory response syndrome appear to accompany this very often fatal condition. Recurrences were very rare. A new subset is highlighted and reviewed in this paper and is termed the ...