Treatment of Catastrophic Antiphospholipid Syndrome

First-Line Treatment Approach

The American College of Cardiology recommends immediate anticoagulation with heparin followed by long-term warfarin (target INR 2.0-3.0) as the foundation of CAPS treatment, which has been associated with improved patient survival in retrospective studies 1, 2
High-dose glucocorticoids should be administered concurrently to address the inflammatory component, as recommended by the Kidney International guidelines 1
Plasma exchange should be initiated promptly and has been associated with improved survival in retrospective studies, according to the Kidney International guidelines 1

The American Thoracic Society recommends that direct oral anticoagulants (DOACs) are specifically contraindicated in antiphospholipid syndrome, especially in triple-positive patients (positive for lupus anticoagulant, anticardiolipin, and anti-β2 glycoprotein-I antibodies) 4, 3
The American College of Chest Physicians recommends vitamin K antagonists (warfarin) as the anticoagulant of choice with a target INR of 2.0-3.0 3, 2
Initiating warfarin therapy should include an overlapping period of parenteral anticoagulation, as recommended by the American College of Chest Physicians 4

Rituximab has shown potential efficacy in case reports and may be considered in refractory CAPS, according to the Kidney International guidelines 1
Emerging evidence supports the use of eculizumab (complement inhibitor) in treatment-resistant cases, targeting complement activation involved in the pathogenesis of tissue injury induced by antiphospholipid antibodies, as reported in the Kidney International journal 1

Using DOACs instead of warfarin is contraindicated as they have been associated with increased thrombotic risk in antiphospholipid syndrome, according to the American College of Chest Physicians and the European Respiratory Society 4, 3
Discontinuing anticoagulation too early - long-term (often indefinite) anticoagulation with warfarin is required, as recommended by the European Respiratory Society and the American College of Cardiology 5, 2