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

نویسندگان

چکیده

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition associated with the development of thrombotic occlusion microvasculature vessels, mortality rate about 50%. The pathogenesis CAPS based on cellular activation, complement system induction, cytokine stimulation, inhibition anticoagulant factors and fibrinolysis, which leads to progressive microangiopathy, disseminated intravascular coagulation (DIC), systemic inflammatory response syndrome. Classification criteria for include microthrombotic involvement ≥3 organs (most commonly lungs, kidneys, central nervous system) ≤1 week high titers antibodies. Differential diagnosis carried out DIC, heparin-induced thrombocytopenia, hemolytic uremic syndrome, HELLP sepsis. Treatment in acute phase involves immunosuppressive therapy (glucocorticoids, plasmapheresis, IV immunoglobulin, rituximab, eculizumab). Timely adequately selected treatment can reduce from 50 30%. Further study needed improve prognosis increase life expectancy patients.

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Current management of catastrophic antiphospholipid syndrome

The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe form and is characterized by clinical evidence of multiple organ involvement developing over a short period of time, histopathological evidence of small vessel occlusions and laboratory confirmation of antiphospholipid antibodies (aPL) as assessed in 2003 in the international consensus statement on the classifica...

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Clinical features and pathogenesis of catastrophic antiphospholipid syndrome.

The concept of antiphospholipid syndrome (APS) has been established (1). It is well-known that one of the clinical features in APSis arterial or venous thrombosis, and that the specificity of so-called antiphospholipid antibodies have diversity against their epitopes, as so-called antiphospholipid antibodies react to phospholipids as well as plasma proteins such as p2-glycoprotein I and prothro...

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[Catastrophic antiphospholipid syndrome: CAPS].

Antiphospholipid syndrome (APS) is well known as an autoimmune thrombotic syndrome with recurrent thromboses. In APS, thromboses occurs both artery and vein, and from large to micro vessels. In contrast, so called catastrophic antiphospholipid syndrome, CAPS, develops multiple thromboses at microvessels mainly within a few weeks and induces to poor prognosis. CAPS often occurs in patients with ...

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ژورنال

عنوان ژورنال: Sovremennaâ Revmatologiâ

سال: 2023

ISSN: ['2310-158X', '1996-7012']

DOI: https://doi.org/10.14412/1996-7012-2023-3-7-15