6.9 Antiphospholipid Syndrome Flashcards

1
Q

Antiphospholipid Syndrome

  • Definition / pathogenesis
  • Clinical Manifestations
    • Venous
    • Arterial
    • Neurologic
    • Pulmonary
    • Cardiac
    • Hematologic
    • GI
    • Adrenal
    • Skin
    • Bone
    • Eyes
A
  • 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
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2
Q

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

A
  • 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
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3
Q

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

A
  • 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
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4
Q

Describe treatment for antiphospholipid syndrome

A
  • 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
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