Catastrophic antiphospholipid syndrome: The current management approach

Best Pract Res Clin Rheumatol. 2016 Apr;30(2):239-249. doi: 10.1016/j.berh.2016.07.004. Epub 2016 Sep 12.


The current recommendation for catastrophic antiphospholipid syndrome (CAPS) management is the standard triple therapy with anticoagulation (AC), glucocorticoids (GCs), plasma exchange (PE), and/or intravenous immunoglobulins (IVIGs). Of note, only AC has a significant effect on the prognosis of these patients. However, from the experimental or basic point of view, there is only indirect evidence to advocate the use of these immunomodulatory therapies (GC, PE, and IVIG) in CAPS. Recently, there have been reports of severe or refractory CAPS patients treated with the monoclonal antibodies rituximab and eculizumab. The first blocks CD20, a surface protein expressed on the cytoplasmic membrane of B cells, and decreases the generation of pathogenic autoantibodies such as antiphospholipid (aPL) antibodies. The second binds with high affinity to C5 complement protein, inhibiting its cleavage and thus preventing the generation of C5b-C9 complex.

Keywords: Anticoagulation; Catastrophic antiphospholipid syndrome; Corticoids; Eculizumab; Intravenous immunoglobulins; Plasma exchange; Review; Rituximab; Sirolimus; Therapeutics.

Publication types

  • Review

MeSH terms

  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Anticoagulants / therapeutic use
  • Antiphospholipid Syndrome / immunology
  • Antiphospholipid Syndrome / therapy*
  • Catastrophic Illness
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Plasma Exchange / methods
  • Prognosis
  • Rituximab / therapeutic use


  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Anticoagulants
  • Glucocorticoids
  • Immunoglobulins, Intravenous
  • Rituximab
  • eculizumab