Antiphospholipid Syndrome – Causes, Clinical Features, Diagnosis & Management

Antiphospholipid Syndrome  (Synopsis & Review Quiz for various Medical PG Entrance Exams)

ANTIPHOSPHOLIPID ANTIBODY SYNDROME

Antiphospholipid antibodies

  • Antibodies against cardiolipin – aCL
  • Antibodies against beta 2 glycoprotein I – B2GPI
  • Lupus anticoagulant – LA
  • Antibodies against phospholipids/cholesterol complexes

 

Clinical manifestations

  • Superficial and deep vein thrombosis, cerebral venous thrombosis, retinal vein thrombosis
  • Signs and symptoms of intracranial hypertension, pulmonary emboli, pulmonary arterial hypertension, and Budd-Chiari syndrome
  • Livedo reticularis
  • Arterial thrombosis – migraines, cognitive dysfunction, transient ischemic attacks, stroke, myocardial infarction, ischemic leg ulcers, digital gangrene, avascular necrosis of bone, retinal artery occlusion, renal artery stenosis, and, infarcts of spleen, pancreas, and adrenals.
  • Premature atherosclerosis – a rare feature of APS
  • Coombs-positive hemolytic anemia and thrombocytopenia
  • Libman-Sacks endocarditis
  • Fetal loss does not appear to be explained by thrombosis, but rather seems to stem from antibody-mediated interference with the growth and differentiation of trophoblasts, leading to a failure of placentation
  • Discontinuation of therapy, major surgery, infection, and trauma may trigger Catastrophic APS

 

Diagnosis: one clinical + one laboratory criterion

Clinical criteria

Laboratory criteria

  • ·         Vascular thrombosis in any tissue or organ
  • ·         Pregnancy morbidity
    • One or more unexplained deaths of a morphologically normal fetus at or beyond the tenth week of gestation
    • One or more premature births of a morphologically normal neonate before the thirty-fourth week of gestation because of eclampsia, severe preeclampsia, or placental insufficiency
    •  A 3 or more unexplained consecutive spontaneous abortions before the 10th  week of  gestation.

 

  • Antiphospholipid antibodies – at intermediate or high titres on two occasions, 12 weeks apart
    • Antibodies against cardiolipin – aCL
    • Antibodies against beta 2
    • glycoprotein I – B2GPI
    • Lupus anticoagulant – LA

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Treatment

  • After the first thrombotic event, patients should be placed on warfarin for life aiming to achieve an INR ranging from 2.5 to 3.5, alone or in combination with 80 mg of aspirin daily.
  • Pregnancy morbidity is prevented by a combination of heparin with aspirin 80 mg
  • Intravenous immunoglobulin 400 mg/kg qd for 5 days may also prevent abortions, while glucocorticoids are ineffective.
  • Aspirin 80 mg daily protects patients with SLE positive for aPL antibodies from developing thrombotic events.

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Antiphopholipid Syndrome

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