What are the treatment options for catastrophic antiphospholipid syndrome (APS)?

Updated: Nov 24, 2020
  • Author: Suneel Movva, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Patients with CAPS are generally very ill, often with active SLE. [30] The condition is too rare to support clinical trials, but improved mortality has been reported with triple therapy consisting of anticoagulation, corticosteroids, and plasma exchange and/or intravenous immunoglobulin. [10] In addition, attention should be paid to associated disorders (eg, infection, SLE).  Cyclophosphamide has been used in cases associated with SLE, although its use in first-trimester pregnancy increases risk of fetal loss. [27, 30] In refractory or relapsing cases, rituximab and eculizumab have been used. [30]


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