Ann Rheum Dis 62:1127 doi:10.1136/ard.2003.006163
  • Leader

Seronegative antiphospholipid syndrome

  1. G R V Hughes,
  2. M A Khamashta
  1. Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London, UK
  1. Correspondence to:
    Dr M A Khamashta

    History repeats itself

    The antiphospholipid syndrome (APS; Hughes syndrome) is now 20 years old.1,2 The clinical features are well defined, and include the tendency to both arterial and venous thrombosis, to recurrent miscarriages, and to occasional thrombocytopenia.

    So too are the features which give the syndrome such a distinctive flavour, setting it apart from other coagulopathies—the severity of the headaches and migraine, the memory loss, the “atypical multiple sclerosis”, the prominence of the livedo reticularis, the heart valve involvement.3

    Traditionally, raised levels of antiphospholipid antibodies (aPL), especially IgG aPL, are associated with the increased thrombotic risk characteristic of the syndrome. However, as always in real clinical practice, there are often discrepancies between antibody levels and clinical disease expression.

    As awareness increases, and the number of patients with APS grows, it comes …