Abstract Number: OC 07.5
Meeting: ISTH 2020 Congress
Theme: Thrombotic Microangiopathies » Antiphospholipid Syndrome
Background: CAPS is a fulminant variant of antiphospholipid syndrome characterized by multi-organ thrombosis and subsequent failure. Traditionally, a combination treatment approach has been adopted which consisted of anticoagulation (AC), glucocorticoids (GC), plasma exchange (PEX) and/or intravenous immunoglobulins (IG). However, CAPS mortality rates remain high despite this approach. Several reports and case series have demonstrated the primary and salvage activity of Rituximab (RTX) and Eculizumab (ECL) in CAPS.
Aims: We conducted this analysis to provide an updated description of the impact of current treatment approaches on the CAPS outcomes.
Methods: We compiled a pooled CAPS database. Frequency tables, Chi-squared tests, and Fisher’s exact tests were used to assess the effect of therapeutic approaches on the clinical outcomes.
Results: Our database consisted of 453 cases. Mortality rates for the following combinations were: 37% for AC/GC, 20% for AC/GC/PEX, 27% for AC/GC/IG, 31% for AC/GC/PEX/IG, 17% for AC/GC/PEX/IG/RTX, and 20% for AC/GC/PEX/IG/RTX/ECL. Mortality of all RTX-based combinations was 17% whereas that of ECL-based combinations was 6%. ECL successfully salvaged RTX failures in 77% of the time. Compared to AC/GC, AC/GC/PEX, RTX-based, and ECL-based combinations had significant lower mortality. When compared to AC/GC/PEX+IG, ECL-based combinations demonstrated significant lower mortality unlike RTX-based combinations. Moreover, when mortality rates were analyzed separately by whether or not the combinations included PEX, IG, or GC, only those that included PEX had a significant lower mortality. However, PEX did not have an impact on mortality in combinations that included RTX and/or ECL.
Conclusions: Our analysis indicates improved CAPS mortality rates when ECL and RTX are incorporated early in the treatment regimen. We recommend a paradigm shift in CAPS frontline treatment which should consist of AC+GC+ECL+RTX+ therapies targeting the underlying medical condition. Time is of the essence, and highly active agents should be used upfront in CAPS to significantly lower mortality and morbidity.
To cite this abstract in AMA style:
Haddad PA, Hammoud D, Gallagher K. The Relative Impact of Treatment Approaches on the Outcome of Catastrophic Antiphospholipid Syndrome (CAPS): Analysis of an Updated Database [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/the-relative-impact-of-treatment-approaches-on-the-outcome-of-catastrophic-antiphospholipid-syndrome-caps-analysis-of-an-updated-database/. Accessed September 27, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/the-relative-impact-of-treatment-approaches-on-the-outcome-of-catastrophic-antiphospholipid-syndrome-caps-analysis-of-an-updated-database/