Review Article

Hughes syndrome (the antiphospholipid syndrome): 25 years old

Authors

C. J. Edwards¹ and G. R. V. Hughes²

1. Department of Rheumatology, Southampton University Hospitals, Southampton, UK
2. London Lupus Centre, London Bridge Hospital, 27-29 Tooley Street, London, SE1 2PR, UK

Received:

18 October 2007

Accepted:

11 December 2007

Published online:

4 March 2008

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Abstract

The antiphospholipid (Hughes) syndrome (APS) is a unique thrombotic disorder, causing both arterial and venous thrombosis, linked to the presence of antibodies directed against phospholipid-protein complexes. The first papers describing the syndrome were published in 1983 and, over the next two years, a series of publications described in detail the various clinical manifestations of the syndrome. Laboratory standardisation workshops were also set up and, in 1984, the first “world” symposium on APS was held. The international APS conferences have continued to grow in numbers and in stature. The APS has already had an impact in obstetrics, in medicine, in psychiatry, and in surgery. The approximate figure of 1 in 5 is a useful guide--1 in 5 of all young strokes, 1 in 5 recurrent miscarriages, 1 in 5 DVTs. More precise data will become available in the worlds of epilepsy, migraine, Alzheimer’s, and MS. The advent of newer “biologic” immunosuppressives such as rituximab may offer help in selected cases. Intravenous immunoglobulin has proved successful, especially in the emergency setting.

Key words

Hughes syndrome - Antiphospholipid syndrome - Thrombosis - Miscarriage