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The antiphospholipid syndrome: a syndrome in evolution.

1992; BMJ; Volume: 51; Issue: 2 Linguagem: Inglês

10.1136/ard.51.2.147

1468-2060

R A Asherson, Ricard Cervera,

Liver Diseases and Immunity

The antiphospholipid syndrome: a syndrome in evolution Since the original description and definition of the 'anti- phospholipid syndrome' (APS)' a number of distinct clinical manifestations related to it have been reported.Most of these may be ascribed to the hypercoagulable state, of which antiphospholipid antibodies seem either to be 'markers' or intimately connected in some way with the highly complex coagulation mechanisms resulting in thrombotic occlusions.Here, we bring these 'expanded' clinical mani- festations together and examine specifically those situations which may now also be included under the broad umbrella of the APS.Although less common than deep vein thrombosis, recurrent fetal loss, or stroke (the well recognised manifesta- tions of the APS), these additional manifestations may occur not only in association with a defined systemic lupus erythematosus (SLE) or 'lupus-like' disease but also in the 'primary' APS.We may further subdivide patients with these lesser known associations into (a) those in whom coagulopathy and thrombosis have been adequately demon- strated as the underlying mechanisms (table 1); and (b) those in whom the association remains unproved or indefinite because no radiological or histopathological evidence of vascular occlusions is available (table 2).Table I Proved associations with thrombosis 1 Hepatic 2 Adrenal