Treatment of Catastrophic Antiphospholipid Syndrome
The treatment of catastrophic antiphospholipid syndrome (CAPS) requires aggressive triple therapy with anticoagulation, glucocorticoids, and plasma exchange, which has been associated with improved patient survival in retrospective studies. 1, 2
First-Line Treatment Approach
Triple Therapy
- Immediate anticoagulation with heparin followed by long-term warfarin (target INR 2.0-3.0) forms the foundation of CAPS treatment 2, 3
- High-dose glucocorticoids should be administered concurrently to address the inflammatory component 1, 4
- Plasma exchange should be initiated promptly and has been associated with improved survival in retrospective studies 1, 5
Important Considerations
- 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) 1, 3
- Vitamin K antagonists (warfarin) remain 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 1
Second-Line and Adjunctive Therapies
For Refractory Cases
- Intravenous immunoglobulins may be considered as an alternative to plasma exchange 4, 5
- Rituximab has shown potential efficacy in case reports and may be considered in refractory CAPS 1, 6
- 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 1, 6
Special Situations
- In patients with concurrent systemic lupus erythematosus (SLE), cyclophosphamide should be considered as part of the treatment regimen 4
- Careful attention to associated diagnoses, such as infections, is critical at the time of diagnosis as these may trigger CAPS 4
Monitoring and Prognosis
- CAPS is associated with high mortality despite aggressive treatment, highlighting the importance of early diagnosis and prompt initiation of therapy 7, 8
- Close monitoring for thrombotic events and treatment response is essential 3
- A multidisciplinary team approach involving rheumatology, hematology, critical care, and nephrology is recommended for optimal management 7
Common Pitfalls to Avoid
- Delaying triple therapy while awaiting complete diagnostic confirmation can increase mortality - treatment should be initiated based on strong clinical suspicion 8
- Using DOACs instead of warfarin is contraindicated as they have been associated with increased thrombotic risk in antiphospholipid syndrome 1, 3
- Discontinuing anticoagulation too early - long-term (often indefinite) anticoagulation with warfarin is required 1, 2
- Underestimating the need for aggressive immunomodulation alongside anticoagulation 4, 5