EFFICACY OF ECULIZUMAB IN CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME: DESCRIPTIVE ANALYSIS FROM THE “CAPS REGISTRY
- Ignasi Rodriguez Pintó, Spain
Abstract
Background and Aims
To assess the efficacy of eculizumab in patients with catastrophic antiphospholipid syndrome (CAPS) based on the real-world experience provided by the “CAPS Registry”.
Methods
We analyzed demographic, clinical and immunological data from all the patients included in the “CAPS Registry” treated with eculizumab and additionally we described eculizumab indication, dose, outcome, use of prophylactic vaccines and adverse effects.
Results
The “CAPS Registry” includes 563 patients from whom 21 (3.7%) were treated with eculizumab (mean age 42.1 years (SD=15); 81.0% female; primary APS patients, 71.4%; precipitating factors, 57.1%): Sixteen (76.1%) of them recovered from the episode of CAPS, 4 (19.0%) patients died, and one (4.7%) patient did not show any improvement with eculizumab. There were no recurrences of thrombosis after a median follow up of 10.7 months. Different regimens were used, but the most common was 900mg weekly for four weeks and 1200mg fortnightly. The required duration of treatment remains unclear.
Conclusions
According to the published evidence and our observations, we consider that eculizumab can be used in addition to the standard treatment as an alternative in patients with CAPS.