Updated: 7/8/2019

Antiphospholipid Syndrome

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Snapshot
  • A 40-year-old woman with systemic lupus erythematosus presents for evaluation of recurrent deep venous thromboses. In the past year, she has had 5 events. On further questioning, she also reveals a history of having 2 spontaneous abortions at > 10 weeks gestation. Today, her physical exam is unremarkable. Blood testing reveals a positive antinuclear antibody and lupus anticoagulant. She is started on anticoagulation.
Introduction
  • Clinical definition
    • an autoimmune disorder, often associated with systemic lupus erythematosus (SLE), characterized by thromboses and recurrent spontaneous abortions
  • Epidemiology
    • incidence
      • increases with age
    • demographics
      • female > male
      • affects adults
    • risk factors
      • SLE
      • drugs
        • procainamide
        • isoniazid
        • hydralazine
        • oral contraceptives
        • malignancy
  • Pathogenesis
    • autoantibodies (lupus anticoagulant, anticardiolipin, and anti-β2 glycoprotein antibodies) react against platelet membranes or prothrombin-platelet membrane complex, activating endothelial cells and platelets
      • this activates complement-mediated thrombosis
  • Associated conditions
    • SLE
    • other autoimmune diseases
    • preeclampsia or eclampsia
  • Prognosis
    • risk of recurrence is high
Presentation
  • Symptoms
    • recurrent fetal loss
    • symptoms related to thrombosis site
      • dyspnea
      • abdominal discomfort
      • neurologic symptoms
      • chest pain
      • skin rash
  • Physical exam
    • cutaneous findings include livedo reticularis
      • reticular (lacy) or mottled violaceous rash
Studies
  • Labs
    • lupus anticoagulant
      • prolonged dilute Russell viper venom time (DRVVT) test
      • prolonged partial thromboplastin time (PTT)
    • anti-β2 glycoprotein (IgG and IgM)
    • anticardiolipin (IgG and IgM)
    • coagulation studies
      • normal or slightly prolonged prothrombin time
      • normal bleeding time
      • normal platelet count
    • false positives due to antibodies
      • + VDRL/RPR due to anticardiolipin antibodies
      • prolonged PTT due to lupus anticoagulant 
        • not corrected by adding normal platelet-free plasma
  • Making the diagnosis
    • based on clinical presentation and laboratory studies
    • diagnostic criteria includes ≥1 clinical and ≥1 laboratory criteria
      • clinical
        • thrombosis
        • spontaneous abortion at ≥ 10 weeks gestational age
        • premature birth at < 35 weeks gestational age due to eclampsia, pre-eclampsia, or placental insufficiency
        • ≥ 3 spontaneous abortions before < 10 weeks gestational age
      • laboratory
        • lupus anticoagulant
        • anticardiolipin antibody
        • anti-β2 glycoprotein
Differential
  • Factor V Leiden mutation
    • distinguishing factor
      • absence of antibodies
  • Antithrombin deficiency
    • distinguishing factor
      • normal PTT
Treatment
  • Medical 
    • warfarin
      • indications
        • for non-pregnant patients
        • prevention of thrombosis
        • target INR 2-3
    • aspirin plus unfractionated heparin
      • indications
        • for pregnant patients 
        • prevention of pregnancy loss
Complications
  • Fetal loss
  • Stroke
 

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(M2.HE.14.1) A 29-year-old woman presents to her primary care doctor for a prenatal visit. She has just learned that she is pregnant and estimates she is approximately 6 weeks pregnant given the timing of her last menstrual cycle. She has a history of recurrent fetal loss. In total, she has had 4 miscarriages between the gestational ages of 8 and 15 weeks. She has a history of systemic lupus erythematosus and has had several DVTs in the past and a clot in her lung once. Her vitals today are unremarkable. She is asking if there are any treatments she should receive to prevent fetal loss. Which of the following is the most appropriate management of this patient? Tested Concept

QID: 104326
1

Aspirin

8%

(5/61)

2

Aspirin and heparin

62%

(38/61)

3

Aspirin and warfarin

2%

(1/61)

4

No treatment indicated

25%

(15/61)

5

Rivaroxaban

2%

(1/61)

M 7 E

Select Answer to see Preferred Response

(M2.HE.14.17) A 27-year-old woman presents to clinic for her annual physical. She is an immigrant and this is her first time seeing this physician. She states she is generally healthy but does have a history of joint pain for which she takes ibuprofen. She has had 4 miscarriages in the past and is trying to get pregnant. Her temperature is 99.2°F (37.3°C), blood pressure is 129/89 mmHg, pulse is 87/min, respirations are 12/min, and oxygen saturation is 98% on room air. Physical exam is notable for the finding in Figure A. Laboratory studies are ordered as seen below.

Serum:
Na+: 138 mEq/L
Cl-: 101 mEq/L
K+: 4.3 mEq/L
HCO3-: 25 mEq/L
BUN: 29 mg/dL
Glucose: 91 mg/dL
Creatinine: 1.7 mg/dL

Which of the following is most likely to be seen in this patient's coagulation parameters?
Tested Concept

QID: 104657
FIGURES:
1

Increased PT

17%

(2/12)

2

Increased PT and PTT

8%

(1/12)

3

Increased PT and PTT, decreased platelets

25%

(3/12)

4

Increased PT, PTT, and D-dimer

42%

(5/12)

5

Increased PTT

8%

(1/12)

M 6 E

Select Answer to see Preferred Response

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