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. Therapeutic plasma exchange (PE) is a procedure of removing and replacing a large volume of plasma with various biologically active substances that have a pathological effect on the patient. Plasma exchange in some diseases is one of the components of pathogenetic therapy (ANCA-associated systemic vasculitis, antiphospholipid syndrome, Goodpasture's syndrome, thrombotic microangiopathy, etc.). PE removes circulating immune complexes damaging tissues and organs and restores the coagulation system. In the practical recommendations of the American Apheresis Society (2016) leading experts in the field of apheresis therapy, based on an analysis of numerous clinical studies, gave clear recommendations to use the apheresis technologies for extracorporeal detoxification. Also 4 categories of indications and contraindications, assessing the benefits of carrying out apheresis procedures for a specific nosology were specified. A number of new diseases have been introduced, such as atopic (neuro) dermatitis (atopic eczema), cardiac neonatal lupus, Hashimoto encephalopathy, HELLP syndrome, in which PE is one of the leading therapeutic approaches as well as cytotoxic and glucocorticosteroid therapy. However, the use of plasma exchange in the treatment of some diseases remains controversial, for example, sepsis. So further research is needed.