Репозиторий Университета

© The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. 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.

© 2018 S. Karger AG.All right reserved. The development of thrombotic microangiopathy (TMA) during pregnancy and in postpartum period requires differential diagnosis between classical obstetric TMA (preeclampsia and HELLP-syndrome) and TMA, for which pregnancy can become a trigger of atypical hemolytic-uremic syndrome (аHUS), thrombotic thrombocytopenic purpura (TTP), obstetric sepsis with DIC-syndrome and catastrophic antiphospholipid syndrome (CAPS) in order to determine treatment strategy. Complement blocking treatment with eculizumab requires aHUS diagnosis, in the pathogenesis of which the main role is played by the uncontrolled activation of the alternative complement pathway. Complement dysregulation occurs in TMAs different from primary aHUS. Short treatment with eculizumab can result in a rapid improvement of patients with secondary aHUS in whom TMA has persisted and renal function worsened despite treatment of the TMA-inducing condition. This observation demonstrates the first experience of successful postpartum catastrophic antiphospholipid syndrome (CAPS) treatment with complement-blocking drug ekulizumab in Russia. Underestimation of the importance of detected laboratory changes and information about a burdened obstetric anamnesis led to the mistaken diagnosis of atypical HUS, which served as the basis for the ekulizumab prescription, without a doubt, saved the patient's life. The role of the complement activation in the pathogenesis of CAPS is discussed. A conclusion is made that the use of complement-blocking therapy in combination with anticoagulants primarily is advised in the obstetric CAPS patients.