Role of β2‐glycoprotein I in the pathogenesis of the antiphospholipid syndrome

Li, J.Y.; Zhu, Xiangrui; Feng, Juan

doi:10.1002/rai2.12072

Cited by 2 publications

(2 citation statements)

References 92 publications

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“…After aβ2GPI binds to its antigen, the conformation of β2GPI changes. The closed conformation of β2GPI transforms into an open conformation ( 28 ). Binding of antibodies to target protein-binding proteins changes their basic functions.…”

Section: Clasification Criteria Of Antiphospholipid Syndromementioning

confidence: 99%

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Antiphospholipid antibodies in patients with antiphospholipid syndrome

Dodig,

Čepelak

2024

Biochem. med. (Online)

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Graphical abstract Highlights • Antiphospholipid syndrome is a rare systemic autoimmune disease characterized by recurrent pregnancy morbidity or thrombosis in combination with the persistent presence of antiphospholipid antibodies in plasma/serum • Specialists in laboratory medicine should take responsibility for the entire analytical process, so that possible interferences are minimized, and physicians obtain reliable results of the patient’s laboratory findings in a timely manner • Due to possible problems in performing tests on aPLs for a more reliable (optimal) interpretation of laboratory findings, a close cooperation between laboratory specialists and clinical specialists is needed Antiphospholipid syndrome (APS) is a rare systemic autoimmune disease characterized by recurrent pregnancy morbidity or thrombosis in combination with the persistent presence of antiphospholipid antibodies (aPLs) in plasma/serum. Antiphospholipid antibodies are a heterogeneous, overlapping group of autoantibodies, of which anti-β2-glycoprotein I (aβ2GPI), anticardiolipin (aCL) antibodies and antibodies that prolong plasma clotting time in tests in vitro known as lupus anticoagulant (LAC) are included in the laboratory criteria for the diagnosis of APS. The presence of LAC antibodies in plasma is indirectly determined by measuring the length of coagulation in two tests - activated partial thromboplastin time (aPTT) and diluted Russell’s viper venom time (dRVVT). The concentration of aβ2GPI and aCL (immunglobulin G (IgG) and immunoglobulin M (IgM) isotypes) in serum is directly determined by solid-phase immunoassays, either by enzyme-linked immunosorbent assay (ELISA), fluoroimmunoassay (FIA), immunochemiluminescence (CLIA) or multiplex flow immunoassay (MFIA). For patient safety, it is extremely important to control all three phases of laboratory testing, i.e. preanalytical, analytical and postanalytical phase. Specialists in laboratory medicine must be aware of interferences in all three phases of laboratory testing, in order to minimize these interferences. The aim of this review was to show the current pathophysiological aspects of APS, the importance of determining aPLs-a in plasma/serum, with an emphasis on possible interferences that should be taken into account when interpreting laboratory findings.

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Section: Clasification Criteria Of Antiphospholipid Syndromementioning

confidence: 99%

“…It is expressed on the surfaces of several cells, e.g. trophoblasts, endothelial cells, monocytes and platelets ( 28 ). It is known that β2GPI exists in at least two different conformations, a closed and an open conformation (90% of the β2GPI circulates in the blood and has a closed conformation).…”

Section: Features Of β2-glycoprotein I and Cardiolipinmentioning

confidence: 99%

Antiphospholipid antibodies in patients with antiphospholipid syndrome

Dodig,

Čepelak

2024

Biochem. med. (Online)

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Antiphospholipid syndrome followed idiopathic retroperitoneal fibrosis in the same patient: A case report and review of literature

Ho,

Li,

Nie

et al. 2024

Rheumatology & Autoimmunity

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IntroductionIdiopathic retroperitoneal fibrosis (IRPF) is a rare fibro‐inflammatory disease characterized by fibrous sclerosis of the retroperitoneal area and may be accompanied by other autoimmune diseases. Herein, we report an unusual case of IRPF with compression of retroperitoneal vessels, then diagnosed with antiphospholipid syndrome (APS), evidenced by multiple venous thrombosis and persistent positivity of antiphospholipid antibodies (aPLs). Along with literature reviews, we highlighted the important of screening for all possible causes in case of thrombosis secondary to IRPF.Case DescriptionA 46‐year‐old male patient was admitted in 2010 due to hypogastralgia and low back pain. The initial laboratory findings showed elevated inflammatory indicators, but negative results of immunology screening. Abdominal computed tomography (CT) revealed diffuse soft tissue in the retroperitoneal area. IRPF was first diagnosed and initially treated with glucocorticoids and immunosuppressants. Along the process of tapering the drugs, IRPF relapsed again. Reexamination of CT scan showed newly developed thrombus in the retroperitoneal vessels. APLs were evaluated and persistently positive with the interval of six months. The diagnosis of APS was confirmed. Glucocorticoids and immunosuppressants were prescribed again accompanied with warfarin. During 17‐month follow‐up periods, there was no relapse of symptoms again.ConclusionWhen IRPF is accompanied by venous thrombosis, in addition to blood flow stasis due to venous compression, other potential causes of thrombophilia should also be considered.

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Role of β2‐glycoprotein I in the pathogenesis of the antiphospholipid syndrome

Cited by 2 publications

References 92 publications

Antiphospholipid antibodies in patients with antiphospholipid syndrome

Antiphospholipid antibodies in patients with antiphospholipid syndrome

Antiphospholipid syndrome followed idiopathic retroperitoneal fibrosis in the same patient: A case report and review of literature

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