akusherstvoObstetrics, Gynecology and ReproductionАкушерство, Гинекология и Репродукция2313-73472500-3194IRBIS LLC10.17749/2070-4968.2015.9.2.032-053akusherstvo-156Research ArticleREVIEW ARTICLEНАУЧНЫЙ ОБЗОРCatastrophic antiphospholipid syndrome. Pathogenesis issuesКАТАСТРОФИЧЕСКИЙ АНТИФОСФОЛИПИДНЫЙ СИНДРОМ. ВОПРОСЫ ПАТОГЕНЕЗАБицадзеВ. О.BitsadzeV. O.

д.м.н., профессор, профессор кафедры акушерства и гинекологии

MD., Professor, Department of Obstetrics and Gynecology

gemostasis@mail.ruХизроеваД. Х.KhizroevaD. K.

к.м.н., ассистент кафедры акушерства и гинекологии

PhD, Department of Obstetrics and Gynecology

gemostasis@mail.ruИдрисоваЛ. Э.IdrisovaL. E.

к.м.н., научный сотрудник кафедры акушерства и гинекологии

PhD, researcher at the Department of of Obstetrics and Gynecology Department

gemostasis@mail.ruАбрамянР. Р.AbramyanR. R.

кандидат медицинских наук кафедры акушерства и гинекологии

PhD, Department of Obstetrics and Gynecology

r_abrahamyan@mail.ruАндрееваМ. Д.AndreevaM. D.

к.м.н., доцент кафедры акушерства, гинекологии и перинатологии

the Chair of Obstetrics, Gynecology and Perinatolology

andreeva_md@mail.ruМакацарияА. Д.MakatsariyaA. D.

д.м.н., член-корреспондент РАН, профессор, заведующий кафедрой акушерства и гинекологии

MD, corresponding member of the Russian Academy of Sciences, Professor,

gemostasis@mail.ruГБОУ ВПО «Первый МГМУ имени И.М. Сеченова» Минздрава России, МоскваРоссияFirst Moscow State Medical Sechenov University of the Ministry of Health Russian FederationRussian FederationГБОУ ВПО «Кубанский государственный медицинский университет» Минздрава России, КраснодарРоссияKuban State Medical University of the Russian Health Ministry, KrasnodarRussian Federation201517062016923253Copyright © Bitsadze V.O., Khizroeva D.K., Idrisova L.E., Abramyan R.R., Andreeva M.D., Makatsariya A.D., 20162016Бицадзе В.О., Хизроева Д.Х., Идрисова Л.Э., Абрамян Р.Р., Андреева М.Д., Макацария А.Д.Bitsadze V.O., Khizroeva D.K., Idrisova L.E., Abramyan R.R., Andreeva M.D., Makatsariya A.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.gynecology.su/jour/article/view/156

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.

Катастрофический антифосфолипидный синдром (КАФС) - наиболее тяжелая форма антифосфолипидного синдрома, проявляющаяся множественными микротромбозами микроциркуляторного русла жизненно важных органов и развитием полиорганной недостаточности на фоне высокого титра антифосфолипидных антител. В обзорной статье приведены международные подходы к терминологии, факторы риска, патогенеза, критерии диагностики, клинические симптомы и методы ведения больных с КАФС. Также представлен собственный опыт ведения 17 пациентов с КАФС. КАФС является состоянием, угрожающим жизни, поэтому требует неотложной терапии. Оптимального лечения КАФС не разработано. КАФС представляет общемедицинскую мультидисциплинарную проблему.

Катастрофическая форма антифосфолипидного синдромаантифосфолипидные антителаосложнения беременностиAntiphospholipid syndromerare diseasethrombotic stormcatastrophic APSReferencesБаркаган З.С., Момот А.П., Сердюк Г.В. Основы диагностики и терапии антифосфолипидного синдрома. М. 2003; 48 с.Barkagan Z.S., Momot A.P., Serdyuk G.V. Basics of diagnostics and therapy of antiphospholipid syndrome. Moscow. 2003; 48. (In Russian).Долгушина Н.В., Макацария А.Д. Вирусные инфекции у беременных: Руководство для врачей. М. 2004; 144 с.Dolgushina N.V., Makatsaria A.D. Viral infections in pregnancy: A Guide for Physicians. Moscow, 2004; 144. (In Russian).Лыдина И.В. Опыт использования бемипарина натрия для повышения результативности экстракорпорального оплодотворения. Тромбоз, гемостаз и реология. 2012; 4 (52).Lydina I.V. Experience in the use of bemiparin sodium to improve the efficiency of in vitro fertilization. Thrombosis, hemostasis and rheology. 2012; 4 (52). (In Russian).Насонов Е.Л. Современные подходы к профилактике и лечению антифосфолипидного синдрома. Тер. Архив. 2003; 5: 83-87.Nasonov E.L. Modern approaches to prevention and treatment of antiphospholipid syndrome. Ter. Archive. 2003; 5: 83-87. (In Russian).Насонов Е.Л. Антифосфолипидный синдром. М. 2004; 440 с.Nasonov E.L. Antiphospholipid syndrome. Moscow. 2004; 440. (In Russian).Макацария А.Д., Бицадзе В.О., Баймурадова С.М., Долгушина Н.В., Юдаева Л.С., Хизроева Д.Х., Акиньшина С.В. Антифосфолипидный синдром – иммунная тромбофилия в акушерстве и гинекологии. М. 2007; 456 с.Makatsariya A.D., Bitsadze V.O., Baimuradova S.M., Dolgushina N.V., Yudaeva L.S., Khizroeva D.Kh., Akin'shina S.V. Antiphospholipid syndrome – the immune thrombophilia in obstetrics and gynecology. Moscow. 2007; 456 s. (In Russian).Хизроева Д.Х. Антифосфолипидные антитела и тромботический шторм. Материалы XIII Всероссийского научного форума «Мать и Дитя». 2011; 213-214.Hizroeva D.H. Antiphospholipid antibodies and thrombotic storm. Proceedings of XIII All- Russian Scientific Forum "Mother and Child". 2011; 213-214. (In Russian).Asherson R.A. The catastrophic antiphospholipid syndrome. J Rheumatol. 1992;19: 508-512.Asherson R.A. The catastrophic antiphospholipid syndrome. J Rheumatol. 1992;19: 508-512.Asherson R.A. Multiorgan failure and antiphospholipid antibodies: the catastrophic antiphospholipid (Asherson's) syndrome. Immunobiology. 2005; 210: 727-733.Asherson R.A. Multiorgan failure and antiphospholipid antibodies: the catastrophic antiphospholipid (Asherson's) syndrome. Immunobiology. 2005; 210: 727-733.Asherson R.A., Cervera R., de Groot P.G., Erkan D., Boffa M.C., Piette J.C., Khamashta M.A., Shoenfeld Y. Catastrophic Antiphospholipid Syndrome Registry Project Group. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis. 2005; 64: 1205-1209.Asherson R.A., Cervera R., de Groot P.G., Erkan D., Boffa M.C., Piette J.C., Khamashta M.A., Shoenfeld Y. Catastrophic Antiphospholipid Syndrome Registry Project Group. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis. 2005; 64: 1205-1209.Asherson R.A., Shoenfeld Y. The role of infection in the pathogenesis of catastrophic antiphospholipid syndrome—molecular mimicry? J Rheumatol. 2000; 27: 12-14.Asherson R.A., Shoenfeld Y. The role of infection in the pathogenesis of catastrophic antiphospholipid syndrome—molecular mimicry? J Rheumatol. 2000; 27: 12-14.Asherson R.A., Espinosa G., Cervera R. Disseminated intravascular coagulation in catastrophic antiphospholipid syndrome: clinical and haematological characteristics of 23 patients. Ann Rheum Dis. 2005; 64: 943-946.Asherson R.A., Espinosa G., Cervera R. Disseminated intravascular coagulation in catastrophic antiphospholipid syndrome: clinical and haematological characteristics of 23 patients. Ann Rheum Dis. 2005; 64: 943-946.Bashiri A., Burstein E., Mazor M. Cerebral palsy and fetal inflammatory response syndrome: a review. J Perinat Med. 2006; 34 (1): 5-12.Bashiri A., Burstein E., Mazor M. Cerebral palsy and fetal inflammatory response syndrome: a review. J Perinat Med. 2006; 34 (1): 5-12.Bernard G.R., Vincent J.L., Laterre P.F., LaRosa S.P., Dhainaut J.F., Lopez-Rodriguez A., Steingrub J.S., Garber G.E., Helterbrand J.D., Ely E.W., Fisher C.J. Jr. Recombinant human protein C Worldwide Evaluation in Severe Sepsis (PROWESS) study group. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Engl J Med. 2001 Mar 8; 344 (10): 699-709.Bernard G.R., Vincent J.L., Laterre P.F., LaRosa S.P., Dhainaut J.F., Lopez-Rodriguez A., Steingrub J.S., Garber G.E., Helterbrand J.D., Ely E.W., Fisher C.J. Jr. Recombinant human protein C Worldwide Evaluation in Severe Sepsis (PROWESS) study group. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Engl J Med. 2001 Mar 8; 344 (10): 699-709.Brenner B., Hoffman R., Carp H., Dulitsky M., Younis J. Live-enox Investigators. Efficacy and safety of two doses of enoxaparin in women with thrombophilia and recurrent pregnancy loss: the Live-enox study. J Thromb Haemost. 2005; 3: 227-229.Brenner B., Hoffman R., Carp H., Dulitsky M., Younis J. Live-enox Investigators. Efficacy and safety of two doses of enoxaparin in women with thrombophilia and recurrent pregnancy loss: the Live-enox study. J Thromb Haemost. 2005; 3: 227-229.Carmona F., Lazaro I., Reverter J.C., Tassies D., Font J., Cervera R., Balasch J. Impaired factor XIIa-dependent activation of fibrinolysis in treated antiphospholipid syndrome gestations developing late-pregnancy complications. Am J Obstet Gynecol. 2006; 194: 457-465.Carmona F., Lazaro I., Reverter J.C., Tassies D., Font J., Cervera R., Balasch J. Impaired factor XIIa-dependent activation of fibrinolysis in treated antiphospholipid syndrome gestations developing late-pregnancy complications. Am J Obstet Gynecol. 2006; 194: 457-465.Cruz M., Fernandez-Alonso A.M., Rodriges I. et al. Prevention of thrombotic complications after cesarean section using two different regimens bemiparin. Medical advice. 2012; 3-7.Cruz M., Fernandez-Alonso A.M., Rodriges I. et al. Prevention of thrombotic complications after cesarean section using two different regimens bemiparin. Medical advice. 2012; 3-7.de Groot P.G., Horbach D.A., Derksen R.H. Protein C and other cofactors involved in the binding of antiphospholipid antibodies: relation to the pathogenesis of thrombosis. Lupus. 1996; 5: 488-493.de Groot P.G., Horbach D.A., Derksen R.H. Protein C and other cofactors involved in the binding of antiphospholipid antibodies: relation to the pathogenesis of thrombosis. Lupus. 1996; 5: 488-493.Erkan D., Leibowitz E., Berman J., Lockshin M.D. Perioperative medical management of antiphospholipid syndrome: hospital for special surgery experience, review of literature, and recommendations. J Rheumatol. 2002 Apr; 29 (4): 843-9.Erkan D., Leibowitz E., Berman J., Lockshin M.D. Perioperative medical management of antiphospholipid syndrome: hospital for special surgery experience, review of literature, and recommendations. J Rheumatol. 2002 Apr; 29 (4): 843-9.Gharavi A.E., Pierangeli S.S., Harris E.N. New developments in viral peptides and APL induction. J Autoimmun. 2000; 15: 227-230.Gharavi A.E., Pierangeli S.S., Harris E.N. New developments in viral peptides and APL induction. J Autoimmun. 2000; 15: 227-230.Haemotology. Clinical cases uncovered. 2009 by Shaun McCann, Robin Foa, Owen Smith, Eibhlin Conneally. Wiley-Blackwell. A John Wiley & Sons, Ltd., Publication. 190 p.Haemotology. Clinical cases uncovered. 2009 by Shaun McCann, Robin Foa, Owen Smith, Eibhlin Conneally. Wiley-Blackwell. A John Wiley & Sons, Ltd., Publication. 190 p.Hemostasis and Thrombosis in obstetrics and gynecology. 2011 by Michael J. Paidas, Nazli Hossain, Tahir S. Shamsi, Marc A. Rodger, Jens Langhoff-Roos, Charles J. Lockwood. Willey-Blackwell. 223 p.Hemostasis and Thrombosis in obstetrics and gynecology. 2011 by Michael J. Paidas, Nazli Hossain, Tahir S. Shamsi, Marc A. Rodger, Jens Langhoff-Roos, Charles J. Lockwood. Willey-Blackwell. 223 p.Karmochkine M., Mazoyer E., Marcelli A., Boffa M.C., Piette J.C. High prevalence of antiphospholipid antibodies in disseminated intravascular coagulation. Thromb Haemost. 1996; 75: 971.Karmochkine M., Mazoyer E., Marcelli A., Boffa M.C., Piette J.C. High prevalence of antiphospholipid antibodies in disseminated intravascular coagulation. Thromb Haemost. 1996; 75: 971.Katzav A., Evert T., Pick C.G., Blank M., Shoenfeld Y., Chapman J. Induction of antiphospholipid syndrome in Factor V Leiden mice. Immunobiology. 2005; 209: 10-11.Katzav A., Evert T., Pick C.G., Blank M., Shoenfeld Y., Chapman J. Induction of antiphospholipid syndrome in Factor V Leiden mice. Immunobiology. 2005; 209: 10-11.Kitchens C.S. Thrombotic storm: when thrombosis begets thrombosis. Am J Med. 1998; 104: 381-385.Kitchens C.S. Thrombotic storm: when thrombosis begets thrombosis. Am J Med. 1998; 104: 381-385.Lai S., Walker D.H., Elghetany M.T. Catastrophic antiphospholipid syndrome: a rare cause of disseminated microvascular thrombotic injury – a case report with pathological and molecular correlative studies. Pathol Int. 2005; 55: 144-149.Lai S., Walker D.H., Elghetany M.T. Catastrophic antiphospholipid syndrome: a rare cause of disseminated microvascular thrombotic injury – a case report with pathological and molecular correlative studies. Pathol Int. 2005; 55: 144-149.Le Thi Thuong D., Tieulie N., Costedoat N., Andreu M.R., Wechsler B., Vauthier-Brouzes D., Aumaitre O., Piette J.C. The Hellp syndrome in the antiphospholipid syndrome: retrospective study of 16 cases in 15 women. Ann Rheum Dis. 2005; 64: 273-278.Le Thi Thuong D., Tieulie N., Costedoat N., Andreu M.R., Wechsler B., Vauthier-Brouzes D., Aumaitre O., Piette J.C. The Hellp syndrome in the antiphospholipid syndrome: retrospective study of 16 cases in 15 women. Ann Rheum Dis. 2005; 64: 273-278.Lu C.S., Horizon A.A., Hwang K.K., FitzGerald J., Lin W.S., Hahn B.H., Wallace D.J., Metzger A.L., Weisman M.H., Chen P.P. Identification of polyclonal and monoclonal antibodies against tissue plasminogen activator in the antiphospholipid syndrome. Arthritis Rheum. 2005; 52: 4018-4027.Lu C.S., Horizon A.A., Hwang K.K., FitzGerald J., Lin W.S., Hahn B.H., Wallace D.J., Metzger A.L., Weisman M.H., Chen P.P. Identification of polyclonal and monoclonal antibodies against tissue plasminogen activator in the antiphospholipid syndrome. Arthritis Rheum. 2005; 52: 4018-4027.Makatsariya A.D., Bitsadze V.O., Khizroeva J.K. Fetal thrombophilia and obstetrics complications. Pathophysiology of haemostasis and thrombosis. Proceeding and abstracts from the 19th International Congress on Thrombosis. Tel-Aviv, Israel, May 140-18, 2006; A10.Makatsariya A.D., Bitsadze V.O., Khizroeva J.K. Fetal thrombophilia and obstetrics complications. Pathophysiology of haemostasis and thrombosis. Proceeding and abstracts from the 19th International Congress on Thrombosis. Tel-Aviv, Israel, May 140-18, 2006; A10.Makatsariya A.D., Asherson R.A., Bitsadze V.O., Akinshina S. Catastrophic antiphospholipid (Asherson's) syndrome and genetic thrombophilic disorders in obstetrics. Autoimmunity Reviews. 2006; Article in press.Makatsariya A.D., Asherson R.A., Bitsadze V.O., Akinshina S. Catastrophic antiphospholipid (Asherson's) syndrome and genetic thrombophilic disorders in obstetrics. Autoimmunity Reviews. 2006; Article in press.Meves S.H, Overbeck U. Endres H.G., Kroguas C., Neubauer H. Dose-dependent effect of early antiplatelet therapy in acute ischaemic stroke. Thrombosis and Haemostasis. 2012; 107: 69-80.Meves S.H, Overbeck U. Endres H.G., Kroguas C., Neubauer H. Dose-dependent effect of early antiplatelet therapy in acute ischaemic stroke. Thrombosis and Haemostasis. 2012; 107: 69-80.Nelson S.M., Scott N., Ian A. Greer. The potential role of heparin in assisted conception. Human Reproduction Update. 2008; 14 (6): 623-645.Nelson S.M., Scott N., Ian A. Greer. The potential role of heparin in assisted conception. Human Reproduction Update. 2008; 14 (6): 623-645.Raschi E., Testoni C., Bosisio D., Borghi M.O., Koike T., Mantovani A., Meroni P.L. Role of the MyD88 transduction signaling pathway in endothelial activation by antiphospholipid antibodies. Blood. 2003; 101: 3495-3500.Raschi E., Testoni C., Bosisio D., Borghi M.O., Koike T., Mantovani A., Meroni P.L. Role of the MyD88 transduction signaling pathway in endothelial activation by antiphospholipid antibodies. Blood. 2003; 101: 3495-3500.Shoenfeld Y. The infectious etiology of APS: Novel cross reactive epitopes on yeasts, bacteria and viruses. 2nd Tutzing Antiphospholipid Conference. Immunobiology. 2005; 209 (S1).Shoenfeld Y. The infectious etiology of APS: Novel cross reactive epitopes on yeasts, bacteria and viruses. 2nd Tutzing Antiphospholipid Conference. Immunobiology. 2005; 209 (S1).Ten Cate H. Pathophysiology of disseminated intravascular coagulation in sepsis. Crit Care Med. 2000; 28: 9-11.Ten Cate H. Pathophysiology of disseminated intravascular coagulation in sepsis. Crit Care Med. 2000; 28: 9-11.The Catastrophic Antiphospholipid Syndrome (Asherson's Syndrome). Monograph. Collected papers; 1992-2005. Printed by Creda Communications. 112 p.The Catastrophic Antiphospholipid Syndrome (Asherson's Syndrome). Monograph. Collected papers; 1992-2005. Printed by Creda Communications. 112 p.Wiedermann F.J., Mayr A., Schobersberger W., Knotzer H., Sepp N., Rieger M., Hasibeder W., Mutz N. Acute respiratory failure associated with catastrophic antiphospholipid syndrome. J Intern Med. 2000; 247: 723-730.Wiedermann F.J., Mayr A., Schobersberger W., Knotzer H., Sepp N., Rieger M., Hasibeder W., Mutz N. Acute respiratory failure associated with catastrophic antiphospholipid syndrome. J Intern Med. 2000; 247: 723-730.Vora S.K., Asherson R.A., Erkan D. Catastrophic antiphospholipid syndrome. J Intensive Care Med. 2006; 21: 144-159.Vora S.K., Asherson R.A., Erkan D. Catastrophic antiphospholipid syndrome. J Intensive Care Med. 2006; 21: 144-159.Villa J., Marco A., Santamaria A. 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.Villa J., Marco A., Santamaria A. 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.Yasuda S., Atsumi T., Matsuura E., Kaihara K., Yamamoto D., Ichikawa K., Koike T. Significance of valine/leucine247 polymorphism of beta2-glycoprotein I in antiphospholipid syndrome: increased reactivity of anti-beta2-glycoprotein I autoantibodies to the valine 247 beta2-glycoprotein I variant. Arthritis Rheum. 2005 Jan; 52 (1): 212-8.Yasuda S., Atsumi T., Matsuura E., Kaihara K., Yamamoto D., Ichikawa K., Koike T. Significance of valine/leucine247 polymorphism of beta2-glycoprotein I in antiphospholipid syndrome: increased reactivity of anti-beta2-glycoprotein I autoantibodies to the valine 247 beta2-glycoprotein I variant. Arthritis Rheum. 2005 Jan; 52 (1): 212-8.

The authors declare that there are no conflicts of interest present.