Antiphospholipid (Hughes) syndrome: a review with update.

Abstract This article reviews the evolution of the concept of Hughes syndrome starting with the discovery of beef heart extract-reactive antibodies in sera of patients with syphilis, presence of such antibodies in some normal individuals who went on to develop systemic lupus erythematosus (SLE), identification of the antigen as anionic phospholipids and some of the cofactors of the blood coagulation cascade, development of more refined techniques for detecting such antibodies and finally its implication in the causation of an acquired hypercoagulable state. The review then discusses the results of extensive international collaborative efforts of the major centres with interest and expertise in Hughes syndrome. It summarizes the recently enunciated classification criteria for this disease. The results and the implications of some of the recent seminal papers, especially on the frequency of different clinical manifestations in a large cohort of patients followed over long-periods, and recommendations for long-term high-intensity anticoagulation for the optimum management of this disease, are also discussed. [ABSTRACT FROM AUTHOR]