References

mrj

Современная ревматология

Modern Rheumatology Journal

1996-70122310-158X

IMA-PRESS, LLC

10.14412/1996-7012-2023-3-7-15

mrj-1425

Research Article

ЛЕКЦИЯ

LECTURE

Катастрофический антифосфолипидный синдром: современные аспекты патогенеза, диагностики и лечения

Catastrophic antiphospholipid syndrome: current aspects of pathogenesis, diagnosis and treatment

https://orcid.org/0000-0002-7410-9784

Клименко

А. А.

Klimenko

A. A.

117997, Москва, ул. Островитянова 1

1, Ostrovitianov Street, Moscow 117997

https://orcid.org/0000-0001-8757-9585

Гаффарова

А. С.

Gaffarova

A. S.

Гаффарова Анифе Севриевна,

117997, Москва, ул. Островитянова 1

Gaffarova Anife Sevrievna,

1, Ostrovitianov Street, Moscow 117997

anife.gaffarova96@gmail.com

https://orcid.org/0000-0001-6890-8777

Демидова

Н. А.

Demidova

N. A.

117997, Москва, ул. Островитянова 1

1, Ostrovitianov Street, Moscow 117997

ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России, МоскваРоссияPirogov Russian National Research Medical University, Ministry of Health of RussiaRussian Federation

2023

10072023

173715

2023

Клименко А.А., Гаффарова А.С., Демидова Н.А.

Klimenko A.A., Gaffarova A.S., Demidova N.A.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://mrj.ima-press.net/mrj/article/view/1425

Катастрофический антифосфолипидный синдром (КАФС) – жизнеугрожающее состояние, связанное с развитием тромботической окклюзии сосудов микроциркуляторного русла, с летальностью около 50%.

В основе патогенеза КАФС лежат клеточная активация, индукция системы комплемента, цитокиновая стимуляция, ингибирование антикоагулянтных факторов и фибринолиза, что приводит к прогрессирующей тромботической микроангиопатии, синдрому диссеминированного внутрисосудистого свертывания (ДВС) и синдрому системного воспалительного ответа. Классификационные критерии КАФС включают микротромботическое поражение ≥3 органов (наиболее часто – легкие, почки и центральная нервная система) в течение ≤1 нед с выявлением антифосфолипидных антител в высоких титрах.

Дифференциальную диагностику проводят с ДВС-синдромом, гепарин-индуцированной тромбоцитопенией, гемолитикоуремическим синдромом, HELLP-синдромом, сепсисом. Лечение КАФС в острой фазе предусматривает антикоагулянтную и иммуносупрессивную терапию (глюкокортикоиды, плазмаферез, внутривенный иммуноглобулин, ритуксимаб, экулизумаб). Своевременная диагностика и адекватно подобранное лечение КАФС позволяют снизить летальность с 50 до 30%.

Необходимо дальнейшее изучение КАФС для улучшения прогноза и увеличения продолжительности жизни больных.

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition associated with the development of thrombotic occlusion of microvasculature vessels, with a mortality rate of about 50%.

The pathogenesis of CAPS is based on cellular activation, complement system induction, cytokine stimulation, inhibition of anticoagulant factors and fibrinolysis, which leads to progressive thrombotic microangiopathy, disseminated intravascular coagulation (DIC), and systemic inflammatory response syndrome. Classification criteria for CAPS include microthrombotic involvement of ≥3 organs (most commonly lungs, kidneys, and central nervous system) for ≤1 week with high titers of antiphospholipid antibodies.

Differential diagnosis is carried out with DIC, heparin-induced thrombocytopenia, hemolytic uremic syndrome, HELLP syndrome, sepsis. Treatment of CAPS in the acute phase involves anticoagulant and immunosuppressive therapy (glucocorticoids, plasmapheresis, IV immunoglobulin, rituximab, eculizumab). Timely diagnosis and adequately selected treatment of CAPS can reduce mortality from 50 to 30%.

Further study of CAPS is needed to improve the prognosis and increase the life expectancy of patients.

катастрофический антифосфолипидный синдромтромбозыакушерские событиямикротромбоз

catastrophic antiphospholipid syndromethrombosisobstetric eventsmicrothrombosis

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