Background Triple therapy (anticoagulation plus corticosteroids plus plasma exchange and/or intravenous immunoglobulins) is empirically prescribed for the management of catastrophic antiphospholipid syndrome (CAPS). However, previous analyses have been inconsistent regarding the beneficial effect of triple therapy on patients' survival.
Objectives The objective of this study was to assess the effect that triple therapy has on the mortality risk of CAPS patients.
Methods In a cohort including 525 episodes of CAPS (CAPS Registry), we evaluated the relationship between triple therapy and mortality. Patients were grouped in three based on their treatments: a) triple therapy (anticoagulation plus corticosteroids plus plasma exchange and/or intravenous immunoglobulins); b) drugs included in the triple therapy but in different combinations; c) none of the treatments included in the triple therapy. The primary endpoint was all-cause mortality. Multivariate logistic regression models were used to compare mortality risk between groups taking into account a set of possible confounding variables.
Results The “CAPS registry” cohort included 525 episodes of CAPS accounting for 502 patients. After excluding 38 episodes (7.2%), a total of 487 episodes of CAPS accounting for 471 patients (mean age 38 years; 67.9% female; primary APS patients 68.8%) were analyzed. Overall, 177 (36.3%) patients died. Triple therapy was prescribed in 197 episodes (40.5%), other combinations in 278 (57.1%), and none of those treatments in 12 episodes (2.5%). According to these three groups, mortality rate increased up to 27.9%, 40.6%, and 75%, respectively. Triple therapy was positively associated with a higher chance of survival when compared to non-treatment (adjusted odds ratio [OR]: 7.7 95%; confidence interval [95CI] 2.0–29.7) or to treatment with other combinations of drugs included in the triple therapy (adjusted OR 6.8; 95CI 1.7–26.9). Triple therapy accounted for a 64% decrease of the risk of death in patients with CAPS that received this combination of drugs.
Conclusions Triple therapy is independently associated to a higher survival rate among CAPS.
Acknowledgements To the CAPS Registry Project Group (European Forum on Antiphospholipid Antibodies).
Disclosure of Interest None declared