6.9 Antiphospholipid Syndrome

Question 1

Antiphospholipid Syndrome

• Definition / pathogenesis
• Clinical Manifestations
  
  • Venous
  • Arterial
  • Neurologic
  • Pulmonary
  • Cardiac
  • Hematologic
  • GI
  • Adrenal
  • Skin
  • Bone
  • Eyes

Answer 1

• Definition / pathogenesis
  
  • Systemic autoimmune d/o
  • Results in venous/arterial thrombosis and/or pregnancy morbidity
  • Signaled by presence of APS antibodies
    
    • Directed against phospholipid binding proteins (cardiolpin and B2-glycoprotein) and lupus anticoagulant
    • Antibodies cause cellular and vascular damage –> blood clots
  • Can occur as primary or secondary dz
    
    • SLE
• Clinical Manifestations
  
  • Venous clots
    
    • DVT, LE > UE
    • Pelvic, renal, pulm, hepatic/portal, axillar/subclav, ocular, cerebral sinuses, IVC
  • Arterial clots
    
    • Cerebral vasculature > stroke/TIA
    • Coronary, retinal, renal, mesenteric
  • Neurologic
    
    • Antiphospholipid antibodies directly attack neuronal tissue
    • Consider in young stroke pt without ovious risk factors
    • Can cause cognitive deficits, a/w white matter lesions on MRI (vasculopathy)
  • Pulmonary
    
    • PE
    • P HTN
    • ARDS
    • Diffuse alveolar hemorrhage
  • Cardiac
    
    • CAD
    • Valve dz
      
      • Mitral > aortic
      • Valvular thickening and nodules –> regurgitant valves
  • Hematologic
    
    • Thrombocytopenia (thrombotic events still happen)
    • Hemolytic anemia
    • Thrombotic microangiopathies
    • Bone marrow necrosis
  • GI
    
    • Ischemia to esophagus, stomach, small bowel, large bowel
    • Bleeding, abd pain, gastric/duodenal ulcers, splenic/panc infarc
    • Hepatic/portal vein thrombosis –> cirrhosis, portal HTN, Budd Chiari
  • Adrenal
    
    • Adrenal insufficiency d/t hemorrhagic infarct
  • Skin
    
    • Livedo reticularis: red/purple lacey reticular patterned rash
    • Cutaneous ulcers
  • Bone
    
    • Osteonecrosis, femoral heads
  • Eyes
    
    • Ocular venous/arterial occlusion
    • Retinal / ischemic optic neuropathy

Question 2

Your pregnant patient wants to know about signs of Antiphospholipid Syndrome. You explain you’d suspect APS if:

Answer 2

• 3 or more consecutive spontaneous abortions at < 10wks gestation
  
  • Not otherwise explained by fetal chromosomal abnormality or maternal anatomy/hormonal cause
• One or ore unexplained abortion of morphologically normal fetus at > 10wks
• One or more premature births < 34wks of morphologically normal neonate
  
  • D/t eclampsia, preeclampsia, placental insufficiency

Question 3

A patient is determined to have APS if they meet what Sapporo Classification Criteria?

Answer 3

• At least one clinical criteria of:
  
  • Vascular thrombosis, or
  • Pregnancy morbidity
• AND at least antiphospholipid antibody on 2 or more occasions at least 12 wks apart of:
  
  • IgG and/or IgM anticardiolipin antibodies
  • IgG and/or IgM anti-B2 glycoprotein I
  • Lupus anticoagulant activity

Question 4

Describe treatment for antiphospholipid syndrome

Answer 4

• Positive aPL tests with NO evidence clinically of APS?
  
  • Treatment NOT recommended
• First line treatment of clinical APS
  
  • AC with heparin vs enoxaparin
  • Bridge to warfarin
  • DOAC not recommended currently
• Second line tx
  
  • Thrombosis prevention is vital given high rates of recurrent thromboticevents
    
    • Venous: warfarin
    • Arterial: warfarin plus ASA for high CV risk patients
    • Address reversible risk factors