Rheumatology Book

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Antiphospholipid Antibody SyndromeAka: Antiphospholipid Syndrome

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  1. Pathophysiology
    1. Recurrent thrombosis of any size vessel
    2. May have no link with SLE despite early associations
  2. Diagnosis
    1. Major clinical associations
      1. Venous thrombosis
        1. Deep Vein Thrombosis
        2. Pulmonary Embolism
      2. Arterial thrombosis
        1. Cerebrovascular Accident (CVA)
        2. Transient Ischemic Attacks
        3. Myocardial Infarction
        4. Gangrene
      3. Recurrent Pregnancy Loss
      4. Thrombocytopenia
      5. Hemolytic Anemia
    2. Other clinical associations
      1. Heart valve abnormality
      2. Positive Coombs tests
      3. Livedo reticularis
      4. Migraine Headaches
      5. Leg ulcers
      6. Myelopathy
      7. Chorea
      8. Pulmonary Hypertension
      9. Avascular necrosis
  3. Lab tests
    1. Autoantibodies are present in 5% of healthy patients
    2. Positive anticardiolipin test (most sensitive test)
      1. IgG > 20 GPL
      2. IgM > 20 MPL units
      3. Possibly IgA positive
    3. Positive Lupus Anticoagulant (most specific test)
    4. Anti-B2-Glycoprotein I Autoantibodies
    5. False positive VDRL (30-40%)
  4. Prevention of thrombotic complications
    1. Anticoagulation
      1. Coumadin
        1. Moderate to high dose (INR >2) is protective
        2. Low dose (INR <1.9) does not prevent thrombosis
      2. Aspirin
        1. Protects against pregnancy loss
        2. Does not protect against thrombosis
    2. Avoid factors predisposing to thrombosis
      1. Oral Contraceptive use
      2. Immobility
    3. Modify atherosclerotic risk factors
      1. Hyperlipidemia
      2. Tobacco abuse
      3. Diabetes Mellitus
  5. References
    1. Levine (2002) N Engl J Med 346:752

Antiphospholipid Syndrome (C0085278)

Definition (MSH)The presence of antibodies directed against phospholipids (ANTIBODIES, ANTIPHOSPHOLIPID). The condition is associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses. In pregnancy it can cause abortion. Of the phospholipids, the cardiolipins show markedly elevated levels of anticardiolipin antibodies (ANTIBODIES, ANTICARDIOLIPIN). Present also are high levels of lupus anticoagulant (LUPUS COAGULATION INHIBITOR).
Definition (CSP)associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses; involves the presence of antibodies directed against phospholipids; cardiolipins show markedly elevated levels of anticardiolipin antibodies; present also are high levels of lupus anticoagulant; in pregnancy it can cause abortion.
ConceptsDisease or Syndrome (T047)
MSHD016736
EnglishAnti Phospholipid Antibody Syndrome, Anti Phospholipid Syndrome, Anti-Phospholipid Antibody Syndrome, Anti-Phospholipid Syndrome, Anticardiolipin syndrome, antiphospholipid antibody syndrome, Antiphospholipid Antibody Syndromes, Antiphospholipid Syndrome, APL - Antiphospholipid syndrome, APS - Antiphospholipid syndrome
Spanishsindrome antifosfolipidico, sindrome antifosfolipido
Parent ConceptsAutoimmune Diseases (C0004364), Syndrome (C0039082), Connective Tissue Diseases (C0009782), Duplicate concept (C1274013)
SourcesCSP, LNC, MSH, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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