Antiphospholipid syndrome – catastrophic

(See separate entry for Antiphospholipid Syndrome)

RecommendationsIVIG is recommended for catastrophic antiphospholipid syndrome, characterized by widespread small vessel thrombosis leading to multiorgan failure.
 
Qualifying Criteria
All of the following criteria must be met:
  • Evidence of rapidly evolving thrombosis involving two or more organs.
  • Unequivocal laboratory evidence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies and/or beta 2 glycoprotein I antibodies).
  • Other causes of thrombotic microangiopathy are considered less likely.
Dose/Frequency of Administration2 g/kg adjusted body weight divided over 2 to 5 days. A single treatment is usually sufficient.

The potential prothrombotic effect of IVIG should be considered in this indication.