VWF, Platelets and the Antiphospholipid Syndrome

Huang, Shengshi; Ninivaggi, Marisa; Chayouâ, Walid; Laat, Bas de

doi:10.3390/ijms22084200

Cited by 23 publications

(10 citation statements)

References 119 publications

(130 reference statements)

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“…The pathogenesis of thrombosis in APS is complex, multifactorial and is still not fully understood [ 25 , 26 , 27 ]. What is clear is that thrombosis is the hallmark of the disease, and that multiple mechanisms involving endothelial cells, platelets, monocytes, neutrophils, and the coagulation and complement pathway are involved.…”

Section: Pathogenesismentioning

confidence: 99%

Neuropsychiatric Manifestations of Antiphospholipid Syndrome—A Narrative Review

Man

Sanna

2022

Brain Sciences

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Antiphospholipid syndrome (APS) is a common autoimmune pro-thrombotic condition characterised by thrombosis and pregnancy morbidity. There are a broad range of neuropsychiatric manifestations associated with APS, from focal symptoms to more global dysfunction. Patients commonly present with transient ischaemic attacks and ischaemic strokes, with identifiable lesions on brain imaging. However, the underlying pathogenesis remains uncertain in other manifestations, such as cognitive dysfunction, seizures, headache and chorea. The aim is to provide a comprehensive review of the various neuropsychiatric manifestations associated with APS. A detailed literature search was applied to PubMed, including citations from 1983 to December 2021.

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Section: Pathogenesismentioning

confidence: 99%

Neuropsychiatric Manifestations of Antiphospholipid Syndrome—A Narrative Review

Man

Sanna

2022

Brain Sciences

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“…Wan et al indicated that LA positivity was an independent associated factor with brain MRI abnormalities (especially lacunae and WMHs) in people without neuropsychiatric symptoms ( 39 ), However, in our study, we tested lupus patients with IgG/IgM aβ 2 -GPI (targeting β 2 -GPI Domain I) and IgG/IgM aCL and did not further investigate IgA aβ 2 -GPI (targeting β 2 -GPI Domain III IV V) or identify β 2 -GPI-dependent aCL IgG. Their diagnostic and clinical significance have been well demonstrated in other studies, which indicates they are correlated with arterial thrombosis ( 26 , 40 , 41 ). This issue will be further explored in our future research.…”

Section: Discussionmentioning

confidence: 90%

Exploring the risk factors for ischemic cerebrovascular disease in systemic lupus erythematosus: A single-center case-control study

Zai-kang

Guan

et al. 2022

Front. Immunol.

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ObjectivesIschemic cerebrovascular disease (ICVD) is one of the most common and severe complications in systemic lupus erythematosus (SLE). We aim to explore the risk factors for ICVD in SLE and to assess their associated clinical characteristics.MethodsIn this study, 44 lupus patients with ICVD (ICVD-SLE) and 80 age- and sex-matched lupus patients without ICVD (non-ICVD-SLE) who were hospitalized in our center between 2014 and 2021 were enrolled. A comprehensive set of clinical and socio-demographic data was recorded. In the ICVD-SLE group, the modified Rankin score (mRS) at 90 days after the occurrence of ICVD, the brain MRI, and arterial ultrasonography findings were collected. Group comparisons were made with continuous variables using an independent t-test or the Mann–Whitney test, and with categorical variables using the chi-square test or Fisher exact test. Multivariate logistic regression analysis was performed to identify the risk factors for ICVD in SLE. Patients with ICVD-SLE were divided into three subgroups according to the gradations of intracranial arterial stenosis (ICAS). The subgroup comparisons were performed by one-way ANOVA test or Kruskal–Wallis test.ResultsOf the 44 patients with ICVD, 45% had a large-vessel ischemic stroke, 50% had a symptomatic lacunar stroke, and 9% had a transient ischemic attack. 2 (4.5%) had both large-vessel ischemic stroke and symptomatic lacunar stroke. Multivariate logistic regression analysis showed that cutaneous vasculitis (OR=7.36, 95% CI=2.11–25.65), anticardiolipin antibody (aCL) (OR=4.38, 95% CI=1.435–13.350), and lupus anticoagulant (LA) (OR=7.543,95% CI=1.789–31.808) were the risk factors, and hydroxychloroquine (HCQ) therapy (OR=0.198, 95% CI=0.078–0.502) was the protective factor, after controlling for confounders. During the analysis of the subgroups, no significant difference was observed between the patients in the group without internal carotid arterial occlusion (ICAS) and those with severe ICAS except for diagnostic delay. However, patients in the moderate ICAS group were older when SLE occurred (P<0.01), had a longer diagnostic delay (P<0.01), a lower percentage of hypocomplementemia (P=0.05) and steroids and HCQ therapy (P=0.01, P=0.05, respectively), a trend toward lower mRS score, but a higher incidence of carotid atherosclerotic plaque (P<0.01), when compared with the other two subgroups.ConclusionCutaneous vasculitis and antiphospholipid antibodies (aPLs) are associated with an increased risk of ICVD, while HCQ therapy may provide protection against ICVD in SLE. The ICVD in younger lupus patients is associated with complement-mediated inflammation and poorer outcome, and require immunosuppressive therapy, whereas the ICVD in elderly patients are characterized by moderate ICAS and carotid atherosclerotic plaques.

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“…In this study we investigated a broad range of specific haemostatic parameters in SARS-CoV-2 PCR positive patients presenting at the emergency department during the first period of the SARS-CoV-2 pandemic. We found that SARS-CoV-2 PCR positive patients had a prothrombotic phenotype as seen by higher fibrinogen and VWF levels [ 25 ], and acquired activated protein C resistance [ 26 ]. One out of seven hospitalized PCR positive patients developed thrombosis, which corresponds well to prevalence of thrombosis in COVID-19 patients reported in other studies [ 27 ].…”

Section: Discussionmentioning

confidence: 99%

Haemostatic differences between SARS-CoV-2 PCR-positive and negative patients at the time of hospital admission

et al. 2022

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Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is associated with thrombosis. We conducted a cohort study of consecutive patients, suspected of SARS-CoV-2 infection presented to the emergency department. We investigated haemostatic differences between SARS-CoV-2 PCR positive and negative patients, with dedicated coagulation analysis. The 519 included patients had a median age of 66 years, and 52.5% of the patients were male. Twenty-six percent of the patients were PCR-positive for SARS-CoV-2.PCR positive patients had increased levels of fibrinogen and (active) von Willebrand Factor (VWF) and decreased levels of protein C and α2-macroglobulin compared to the PCR negative patients. In addition, we found acquired activated protein C resistance in PCR positive patients. Furthermore, we found that elevated levels of factor VIII and VWF and decreased levels of ADAMTS-13 were associated with an increased incidence of thrombosis in PCR positive patients. In conclusion, we found that PCR positive patients had a pronounced prothrombotic phenotype, mainly due to an increase of endothelial activation upon admission to the hospital. These findings show that coagulation tests may be considered useful to discriminate severe cases of COVID-19 at risk for thrombosis.

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VWF, Platelets and the Antiphospholipid Syndrome

Cited by 23 publications

References 119 publications

Neuropsychiatric Manifestations of Antiphospholipid Syndrome—A Narrative Review

Neuropsychiatric Manifestations of Antiphospholipid Syndrome—A Narrative Review

Exploring the risk factors for ischemic cerebrovascular disease in systemic lupus erythematosus: A single-center case-control study

Haemostatic differences between SARS-CoV-2 PCR-positive and negative patients at the time of hospital admission

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