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The effect of triple therapy on the mortality of catastrophic anti-phospholipid syndrome patients

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Rodríguez-Pintó I1, Espinosa G1, Erkan D2, Shoenfeld Y3, Cervera R1; CAPS Registry Project Group. Rheumatology (Oxford). 2018 Apr 11. doi: 10.1093/rheumatology/key082. [Epub ahead of print]


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1 Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Spain.

2 Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, New York, NY, USA.

3 Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Aviv, Israel.


OBJECTIVES: The objective of this study was to assess the effect that triple therapy (anticoagulation plus CS plus plasma exchange and/or IVIGs) has on the mortality risk of patients with catastrophic APS (CAPS) included in the CAPS Registry.

METHODS: Patients from the CAPS Registry were grouped based on their treatments: triple therapy; drugs included in the triple therapy but in different combinations; and 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.

RESULTS: The CAPS Registry cohort included 525 episodes of CAPS accounting for 502 patients. After excluding 54 episodes (10.3%), a total of 471 patients with CAPS were included [mean (s.d.) age 38.5 years (17); 68.2% female; primary APS patients 62%]. Overall, 174 (36.9%) patients died. Triple therapy was prescribed in 189 episodes (40.1%), other combinations in 270 (57.3%) and none of those treatments in 12 episodes (2.5%); the mortality rate in the three groups was 28.6, 41.1 and 75%, respectively. Triple therapy was positively associated with a higher chance of survival when compared with non-treatment [adjusted odds ratio (OR) = 9.7, 95% CI: 2.3, 40.6] or treatment with other combinations of drugs included in the triple therapy (adjusted OR = 1.7, 95% CI: 1.2, 2.6). No statistical differences were found between patients that received triple therapy with plasma exchange or IVIGs (P = 0.92).

CONCLUSION: Triple therapy is independently associated with a higher survival rate among patients with CAPS.