Is there a microangiopathic antiphospholipid syndrome?

摘要

Revealing the evolution of the term APS and its commonalities with other microangiopathic disorders The occurrence of small-vessel occlusions (thrombotic microangiopathy) in association with anti-phospholipid antibodies (aPL) affecting, for example, the retinal vessels, the nail fold,the skin, or major intrabdominal organs such as the kidney, the liver or the bowel,5 although uncommon, is well documented. These occlusions have been described in the simple or classic antiphospholipid syndrome (APS), whether or not associated with systemic lupus erythematosus (SLE), or in the primary APS, but they do not in any way dominate the clinical picture in these conditions. However, with the description and definition of the catastrophic APS (also known as Asherson's syndrome) in 1992 (a new subset of the APS, often fatal, with many distinguishing characteristics separating it from the simple APS), there has been renewed interest in the thrombotic micro-angiopathies and their association with aPL. Although large-vessel occlusions do occur in catastrophic APS, they do not dominate the clinical picture, and their frequency is completely different from that encountered in the classic APS itself. Additionally, the catastrophic APS is frequently accompanied by a systemic inflammatory response syndrome (SIRS).