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

Ignasi Rodríguez-Pintó; Gerard Espinosa; Doruk Erkan; Yehuda Shoenfeld; Ricard Cervera; CAPS Registry Project Group

doi:10.1093/rheumatology/key082

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

Rheumatology (Oxford). 2018 Jul 1;57(7):1264-1270. doi: 10.1093/rheumatology/key082.

Authors

Ignasi Rodríguez-Pintó¹, Gerard Espinosa¹, Doruk Erkan², Yehuda Shoenfeld³, Ricard Cervera¹; CAPS Registry Project Group

Collaborators

CAPS Registry Project Group:
R Cervera, G Espinosa, I Rodríguez-Pintó, Y Shoenfeld, D Erkan, J C Piette, M Jacek, B Roca, M Tektonidou, H Moutsopoulos, J Boffa, J Chapman, L Stojanovich, M P Veloso, S Praprotnik, B Traub, R Levy, T Daryl, Tan Daryl, M C Boffa, A Makatsaria, M Ruano, A Allievi, W You, M Khamastha, S Hughes, J Menendez Suso, J Pacheco, M F Boriotti, C Dias, G Pangtey, S Miller, S Policepatil, L Larissa, S Marjatta, S Carolyn, T Noortje, K Reiner, S Arteaga, T Leilani, D Langsford, M Niedzwiecki, V Queyrel, R Moroti-Constantinescu, C Romero, K Jeremic, A Urbano, R Hurtado-García, A Kumar Das, N Costedoat-Chalumeau, F Yngvar, J A Gomez-Puerta, E de Meigs, J P Smith, E Zakharova, A Nayer, W Douglas, R Lyndsey, V Blanco, C Vicent, K Natalya, L Damian, E Valentini, B Giula, M Casal Moura, O Araújo Loperena, Y Ritter Susan, G Guettrot Imbert, H Almasri, T Hospach, B Mouna, A Robles, H Wilson, P Guisado, R Ruiz, J Rodriguez

Affiliations

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.

PMID: 29660074
DOI: 10.1093/rheumatology/key082

Abstract

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.

Keywords: antiphospholipid syndrome; catastrophic antiphospholipid syndrome; immunoglobulins; mortality; plasma exchange; survival rate; systemic lupus erythematosus; treatment; triple therapy.