Catastrophic antiphospholipid syndrome. Pathogenesis issues

V. O. Bitsadze; D. K. Khizroeva; L. E. Idrisova; R. R. Abramyan; M. D. Andreeva; A. D. Makatsariya

doi:10.17749/2070-4968.2015.9.2.032-053

Catastrophic antiphospholipid syndrome. Pathogenesis issues

V. O. Bitsadze, D. K. Khizroeva, L. E. Idrisova, R. R. Abramyan, M. D. Andreeva, A. D. Makatsariya

https://doi.org/10.17749/2070-4968.2015.9.2.032-053

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Abstract

Сatastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome that results in a widespread coagulopathy and high titres os antiphospholipid antibodies and affects predominantly small vessels supplying organs with the development of multiorgan failure. International approaches on terminology, risk factors, pathogenesis, criteria of diagnostics, clinical symptoms and management of patients with CAPS are reviewed. Own clinical experience of management 17 patients with CAPS development also presented. CAPS is life-threatening condition but optimal treatment for CAPS is not developed yet. CAPS presents a multidisciplinary problem.

Keywords

Antiphospholipid syndrome, rare disease, thrombotic storm, catastrophic APS

About the Authors

V. O. Bitsadze

First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
MD., Professor, Department of Obstetrics and Gynecology

D. K. Khizroeva

First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
PhD, Department of Obstetrics and Gynecology

L. E. Idrisova

First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
PhD, researcher at the Department of of Obstetrics and Gynecology Department

R. R. Abramyan

First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
PhD, Department of Obstetrics and Gynecology

M. D. Andreeva

Kuban State Medical University of the Russian Health Ministry, Krasnodar
Russian Federation
the Chair of Obstetrics, Gynecology and Perinatolology

A. D. Makatsariya

First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
MD, corresponding member of the Russian Academy of Sciences, Professor,

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For citation:

Bitsadze V.O., Khizroeva D.K., Idrisova L.E., Abramyan R.R., Andreeva M.D., Makatsariya A.D. Catastrophic antiphospholipid syndrome. Pathogenesis issues. Obstetrics, Gynecology and Reproduction. 2015;9(2):32-53. (In Russ.) https://doi.org/10.17749/2070-4968.2015.9.2.032-053