Rheumatology Book


Antiphospholipid Antibody Syndrome

Aka: Antiphospholipid Antibody Syndrome, Antiphospholipid Syndrome, Lupus Anticoagulant Disorder
  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 PositiveVDRL (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 346:752-63 [PubMed]

Antiphospholipid Syndrome (C0085278)

Definition (NCI) A syndrome associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses, marked by the presence of antibodies directed against phospholipids.
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.
Concepts Disease or Syndrome (T047)
MSH D016736
ICD10 D68.61
SnomedCT 201450008, 26843008, 19267009
LNC LP40278-1, LA15309-0
English Anti Phospholipid Syndrome, Anti-Phospholipid Syndrome, Syndrome, Anti-Phospholipid, Syndrome, Antiphospholipid, Anti-Phospholipid Antibody Syndrome, Antiphospholipid Antibody Syndrome, Anti Phospholipid Antibody Syndrome, Antibody Syndrome, Anti-Phospholipid, Antibody Syndrome, Antiphospholipid, Antiphospholipid Antibody Syndromes, Syndrome, Anti-Phospholipid Antibody, Syndrome, Antiphospholipid Antibody, APL - Antiphospholipid syndrom, APS-Antiphospholipid syndrome, antiphospholipid syndrome (diagnosis), antiphospholipid syndrome, Antiphospholipid Syndrome [Disease/Finding], Antiphospholipid Syndrome, anti phospholipids syndrome, anticardiolipin syndrome, anti phospholipid syndrome, anti-phospholipid syndrome, antiphospholipids syndrome, antiphospholipid antibody syndrome, Antiphospholipid antibody syndrome, Lupus Anticoagulant Disorder, Hughes Syndrome, Syndrome, Hughes, Antiphospholipid syndrome, APL - Antiphospholipid syndrome, APS - Antiphospholipid syndrome, Anticardiolipin syndrome, Antiphospholipid syndrome (disorder), syndrome; anticardiolipin, syndrome; antiphospholipid, anticardiolipin; syndrome, antiphospholipid; syndrome
Italian Sindrome da antifosfolipidi, Sindrome da anticorpi antifosfolipidici, Sindrome antifosfolipidica
Swedish Antifosfolipidsyndrom
Japanese コウリンシシツコウタイショウコウグン, 抗リン脂質抗体症候群, 抗りん脂質症候群, 抗りん脂質抗体症候群, 抗リン脂質症候群, 抗燐脂質抗体症候群, 抗燐脂質症候群, 燐脂質抗体症候群
Czech syndrom antifosfolipidových protilátek, antifosfolipidový syndrom, Antifosfolipidový syndrom
Finnish Fosfolipidivasta-aineoireyhtymä
Spanish síndrome antifosfolípido (trastorno), síndrome antifosfolipídico, síndrome antifosfolipídico (trastorno), síndrome antifosfolípido, Síndrome de los Anticuerpos Antifosfolípidos, Síndrome Antifosfolipidico, Síndrome del Anticuerpo Antifosfolípido, Síndrome antifosfolípido, Síndrome Antifosfolípido
Polish Zespół antyfosfolipidowy, APS
Hungarian Antiphospholipid syndroma
Norwegian Antifosfolipidsyndrom
Portuguese Síndrome Antifosfolípide, Síndrome do Anticorpo Antifosfolípide, Síndrome dos Anticorpos Antifosfolípides, Síndrome antifosfolipídica, Síndrome Antifosfolipídica
Dutch anticardiolipine; syndroom, antifosfolipide; syndroom, syndroom; anticardiolipine, syndroom; antifosfolipide, antifosfolipidensyndroom, Antifosfolipidesyndroom, Syndroom, antifosfolipide-
French Syndrome des anti-phospholipides, Syndrome de Soulier, Syndrome des anticorps antiphospholipides (SAPL), Syndrome des anticorps antiphospholipides, Syndrome des antiphospholipides
German Antiphospholipidsyndrom, Antiphospholipid-Syndrom, Phospholipid-Antikörper-Syndrom
Derived from the NIH UMLS (Unified Medical Language System)

Lupus anticoagulant disorder (C0311370)

Concepts Disease or Syndrome (T047)
ICD10 D68.62
SnomedCT 19267009
English lupus anticoagulant, lupus anticoagulant disorder, lupus anticoagulant disorder (diagnosis), Lupus anticoagulant disorder, Lupus anticoagulant syndrome, lupus anticoagulant syndrome, Lupus anticoagulant, Lupus anticoagulant inhibitor syndrome, SLE inhibitor syndrome, Lupus anticoagulant disorder (disorder)
Spanish anticoagulante lúpico (trastorno), anticoagulante lúpico, síndrome de inhibidor LES, síndrome de inhibidor anticoagulante lúpico
Derived from the NIH UMLS (Unified Medical Language System)

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