Hughes syndrome (the antiphospholipid syndrome): a disease of our time.
A pro-thrombotic condition was described in 1983 which was characterised by the presence of circulating antiphospholipid antibodies, as well as peripheral thrombosis (e.g. DVT), a tendency to internal organ involvement, repeated miscarriage, and, occasionally, thrombocytopenia (aPL) (Hughes, Br Med J 287:1088-1089, 1983). Previously, there had been a number of observations, mainly in patients with lupus having "false positive" tests for syphilis, miscarriage and circulating lupus anticoagulants. The description in 1983 had three notable features (a) a detailed comprehensive clinical picture of the syndrome; (b) this description differed from other coagulopathies in showing a propensity for arterial thrombosis (e.g. stroke and heart attack); and (c) this was a syndrome quite independent from lupus. There are indications that the primary antiphospholipid syndrome will turn out to be more common than lupus, though this could still be a reflection of referral practice.
The London Lupus Centre, London Bridge Hospital, London, UK. graham.hughes@hcaconsultant.co.uk
Graham R V
GR
Hughes
D000022:Abortion, Spontaneous / Q000209:etiology / Q000628:therapy; D017152:Antibodies, Antiphospholipid / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D001157:Arterial Occlusive Diseases / Q000150:complications; D001241:Aspirin / Q000627:therapeutic use; D001927:Brain Diseases / Q000209:etiology; D002318:Cardiovascular Diseases / Q000209:etiology; D003251:Constriction, Pathologic / Q000150:complications; D005260:Female; D006495:Heparin, Low-Molecular-Weight / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007238:Infarction / Q000209:etiology; D011247:Pregnancy; D013927:Thrombosis / Q000150:complications; D014859:Warfarin / Q000627:therapeutic use
D017152:Antibodies, Antiphospholipid; D006495:Heparin, Low-Molecular-Weight; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D014859:Warfarin; D001241:Aspirin