What is the best strategy in treating catastrophic antiphospholipid syndrome?

The current recommendation for specific therapy of catastrophic antiphospholipid syndrome (CAPS) is the triple therapy with anticoagulation, glucocorticoids, plasma exchange and/or intravenous immunoglobulins. Of note, only anticoagulation had a significant effect improving the vital prognosis of these patients. From the experimental point of view, there is only indirect evidence to advocate the use of these immunomodulatory therapies in CAPS. Recently, two monoclonal antibodies, rituximab and eculizumab, have been successfully used in some cases of severe or refractory CAPS. The first decreases the generation of pathogenic autoantibodies such as antiphospholipid. The second prevents the generation of C5b-C9 complex. The current review describes the conventional and recent modalities of CAPS treatment, discussing the rationale for each one.