Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology

Author:

Makatsariya Alexander D.1,Khizroeva Jamilya2,Bitsadze Viktoriya O.2

Affiliation:

1. Correspondent Member of the Russian Academy of Sciences , Head of the Chair of Obstetrics and Gynecology of Medical Prophylaxis Faculty of I.M. Sechenov First Moscow State Medical University, Vice-President of Russian Association of Obstetricians and Gynaecologists , Dmitrii Ulyanov Street 4-2-432 , 119333 Moscow , Russia , Tel.: +7(903)728-0897

2. Professors of the Chair of Obstetrics and Gynecology of Medical Prophylaxis Faculty of I.M. Sechenov First Moscow State Medical University , Zemlyanoi Val, 62 , 109004 Moscow , Russia

Abstract

Abstract Background: Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called “thrombotic storm” and multiple organ failure, that is, CAPS. Materials and methods: Since 2001–2016, we discovered 17 patients with CAPS development. Conclusion: CAPS is life-threatening condition, but optimal treatment for CAPS is not developed yet and the mortality rate is as high as 30%–40%.

Publisher

Walter de Gruyter GmbH

Subject

Obstetrics and Gynecology,Pediatrics, Perinatology and Child Health

Reference35 articles.

1. Antiphospholipid antibodies and thrombotic storm. J. Khizroeva. Proceedings of XIII All-Russian Scientific Forum “Mother and Child”. 2011, pp. 213–4.

2. Villa J, Marco A, Santamaria A, Mateo J, Simu M, Constans M, et al. Haemostasis and Thrombosis Unit, Haematology Service. Gynaecology and Obstetrics Department, Hospital de la Santa Creu I Sant Pau, Barcelona, Spain. 2006. The 2nd International symposium Women’s health issues in thrombosis and Haemostasis. Budapest, Hungary, Feb 4–6, 2005.

3. Asherson RA. Multiorgan failure and antiphospholipid antibodies: the catastrophic antiphospholipid (Asherson’s) syndrome. Immunobiology. 2005;210:727–33.

4. Walsh N. Clinical picture still emerging in CAPS registry. Ob Gyn News. 2006;41:25.

5. Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, et al. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis. 2005;64:1205–9.

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