Antiphospholipid (Hughes') syndrome

摘要

The antiphospholipid syndrome, first described in 1983, is now recognised as an important prothrombotic disorder associated with a specific group of antibodies. Its main clinical feature is thrombosis, both venous and arterial (especially recurrent cerebral ischaemic attacks). Other features include mild thrombocytopenia, chorea, heart valve disease, livedo reticularis, and, most commonly, recurrent pregnancy loss. The importance of the syndrome in general medicine, especially in vascular and neurological disease, is now acknowledged. The syndrome has had various names. Hughes originally studied it in patients with systemic lupus ery-thematosus but recognised that most patients "had atypical lupus, or no lupus at all"—hence the concept of "primary" antiphospholipid syndrome. In the early 1990s it was found that a phospholipid binding protein, β_2 glycoprotein I, was required for the binding of antibodies to phospholipids. More recently, other such proteins have been shown to be involved. The complex and possibly indirect links between antiphospholipid antibodies and thrombosis led to the suggestion that the name of the syndrome be changed to "Hughes' syndrome."