Dr Shubham Upadhyay

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OVERVIEW
• Introduction
• Epidemiology
• Pathogenesis
• Clinical Manifestations
• Diagnosis
• Differential Diagnosis
• Treatment

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INTRODUCTION
• Definition
• Autoantibody mediated acquired thrombophilia
• Recurrent arterial and/or venous thrombosis and/or pregnancy morbidity
• Females
• Primary vs Secondary
• Catastrophic APS
• Autoantibodies:
• Against PL and/or PL binding plasma proteins
ü Cardiolipin, Phosphocholine, Phosphatidyserine
ü Beta 2 Glycoprotein I
ü Lupus Anticoagulant
• Biological False Positive Serologic Test for Syphilis
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EPIDEMIOLOGY
• 5 per 1 lac cases per year

• Anti PL antibodies occur in 1-5% of general populaton

• >2/3rd of SLE patients possess these Ab

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PATHOGENESIS
• Initiating Event - Infections, Oxidative stress, Physical stress (Surgery
or trauma)

• Binding of anti-PLs to disrupted endothelial cells --> Intravascular

Coagulation --> Thrombus Formation

• Complement Activation

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CLINICAL FEATURES

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CLINICAL FEATURES

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CLINICAL FEATURES

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DIAGNOSIS
• <55 ysrs, Pregnancy morbidity, Livedo Reticularis, thrombocytopenia
• Diagnosis: 1 Clinical Criteria + 1 Laboratory Criteria
Clinical Criteria Laboratory Criteria
1. Vascular Thrombosis (one or more clinical 1. Lupus Anticoagulant (LA)
episodes of arterial, venous or small vessel
thrombosis in any tissu or organ) 2. Anticardiolipin )aCL) and/or
2. Pregnancy Morbidity
a. ≥1 unexplained death of a morphologically 3. Anti- β2GPI antibodies
normal fetus at or beyond 10 weeks of gestation
b. ≥1 premure births of a morphologically
normal neonate before 34 weeks o gestation due to At intermediate or high titers on
eclampsia, severe pre eclampsia or placental two occasions, 12 weeks apart
insufficiency
c. ≥3 unexplained consecutive spontaneous
abortions before 10th week of gestation

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DIFFERENTIAL DIAGNOSIS
• Exclusion of other inherited or acquired casesof thrombophilia,
Coombs positive hemoytic anemia, and thrombocytopenia

• Livedo Reticularis
• Atherosclerosis
• PAN
• SLE
• Cryoglobulinemia
• Myeloproliferative Disorders
• Hypercholesterolemia

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TREATMENT
• After 1st thrombotic event
• Warfarin - Target INR 2.5 to 3.5
• Aspirin 80 mg daily
• Pregnancy Morbidity Prevention
• LMWH + Aspirin 80 mg daily
• IVIg 400 mg/kg for 5 days
• Patient with aPL without any clinical event: Risk of Thrombosis
• Aspirin 80 mg daily + HCQ 200 mg daily
• CAPS
• IVIg, LMWH
• For HIT: Fondaparinux 7.5 mg sc daily or Rivaroxaban 10 mg OD
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