Management of refractory cases of catastrophic antiphospholipid syndrome.

The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. Refractory catastrophic APS may be defined as patients who did not respond to first-line therapies (anticoagulation, glucocorticoids and plasma exchange and/or intravenous immunoglobulins) and died in the acute phase of the episode or patients with recurrent episodes of catastrophic APS. The purpose of this review is to focus on the current management of these refractory patients and some of the potential new therapeutic approaches.