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Antiphospholipid Antibody-negative Syndrome — Other Phospholipids

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Hughes Syndrome

Abstract

Literally speaking, an antiphospholipid antibody (aPL)-negative antiphospholipid syndrome (APS) is an oxymoron, given that aPL positivity is considered to be a sine qua non of APS. Nonetheless, the term has acquired meaning and refers to patients with clinical manifestations of APS, who are thought to have the syndrome despite negative results in conventional aPL tests (anticardiolipin and lupus anticoagulant assays). There are several possible rationales for including such patients under the rubric of APS. Conceptually, the most important explanation, and the focus of this chapter, is laboratory evidence of autoantibodies thought to be associated with APS, but not detected in conventional aPL assays. These include autoantibodies to certain phospholipid-binding plasma proteins, as well as antibodies detected in immunoassays using phospholipids other than cardiolipin. A second possibility suggested by several reports is that conventional aPL tests may be transiently decreased or negative during an acute episode of thrombosis, presumably due to tissue deposition of circulating antibodies [1, 2]. More trivial explanations include inadequacies in the performance of conventional aPL assays, e.g., use of an insensitive lupus anticoagulant assay, and incomplete use of conventional assays, e.g., failure to test for IgA anticardiolipin antibodies in selected patients.

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Authors
  1. R. A. S. Roubey
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Editors and Affiliations

  1. Lupus Research Unit, St Thomas’ Hospital, Lambeth Palace Road, SE1 7EH, London, UK

    M. A. Khamashta MD, MRCP, PhD (Deputy Head) (Deputy Head)

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© 2000 Springer-Verlag London

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Roubey, R.A.S. (2000). Antiphospholipid Antibody-negative Syndrome — Other Phospholipids. In: Khamashta, M.A. (eds) Hughes Syndrome. Springer, London. https://doi.org/10.1007/978-1-4471-3666-8_26

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  • DOI: https://doi.org/10.1007/978-1-4471-3666-8_26

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-85233-232-7

  • Online ISBN: 978-1-4471-3666-8

  • eBook Packages: Springer Book Archive

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