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[Antiphospholipid syndrome].

K Takabayashi1

  • 1Second Department of Internal Medicine, Chiba University, School of Medicine.

Nihon Rinsho. Japanese Journal of Clinical Medicine
|March 17, 1999
PubMed
Summary
This summary is machine-generated.

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Antiphospholipid Syndrome (APS), or Hughes syndrome, involves antibodies targeting beta 2-GPI, not cardiolipin. This autoimmune disorder can cause thrombosis, atherosclerosis, and severe symptoms, including rare catastrophic cases.

Area of Science:

  • Immunology
  • Rheumatology
  • Vascular Medicine

Context:

  • Antiphospholipid Syndrome (APS), also known as Hughes syndrome, is an autoimmune disorder.
  • Recent research redefines the antigen for anticardiolipin antibodies (aCL).
  • Lupus Anticoagulant may be associated with anti-prothrombin antibodies.

Purpose:

  • To review current understanding of Antiphospholipid Syndrome (APS).
  • To highlight recent findings on aCL antigenicity and pathogenic antibodies.
  • To discuss the expanded spectrum of APS symptoms and rare variants.

Summary:

  • Anticardiolipin antibody (aCL) targets beta 2-glycoprotein I (beta 2-GPI) when bound to cardiolipin or oxidized, not cardiolipin itself.
  • Lupus Anticoagulant is now potentially identified as an anti-prothrombin antibody.

Related Experiment Videos

  • Clinical manifestations include livedo, Sneddon syndrome, pulmonary hypertension, skin ulcers, accelerated atherosclerosis, and thrombosis.
  • Both IgA aCL and IgG aCL are implicated in thrombosis.
  • Catastrophic APS is a rare but frequently fatal variant.
  • Impact:

    • Advances understanding of APS pathogenesis and antibody targets.
    • Expands the recognized clinical features and diagnostic considerations for APS.
    • Highlights the thrombotic and potentially atherosclerotic risks associated with APS.
    • Emphasizes the severity of rare catastrophic APS cases.