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A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Biochemistry".

Deadline for manuscript submissions: closed (10 April 2023) | Viewed by 16868

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Special Issue Editors

Prof. Dr. Cristina Belizna

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Guest Editor

Vascular and Coagulation Department, University Hospital Angers, 49100 Angers, France
Interests: antiphospholipid syndrome; OMICS; immune; autoimmune; rheumatology
Special Issues, Collections and Topics in MDPI journals

Prof. Dr. Ljudmila Stojanovich

E-Mail Website
Co-Guest Editor

University Medical Center, Belgrade University, Belgrade, Serbia
Interests: systemic lupus erythematosus; antiphospholipid syndrome; vaccination patients with auto-immune inflammatory rheumatic diseases

Dr. Jaume Alijotas-Reig

E-Mail Website
Co-Guest Editor

Department of Medicine, Autonomous University of Barcelona, 08193 Barcelona, Spain
Interests: antiphospholipid antibodies

Special Issue Information

Dear Colleagues,

We are pleased to kindly invite you to contribute with your research to the special Issue: Antiphospholipid Syndrome.

Antiphospholipid syndrome is an autoimmune systemic disorder characterized by arterial, venous, or small vessel thrombosis and/or recurrent early pregnancy loss, fetal loss, or pregnancy morbidity in the setting of documented persistent anti-phospholipid antibodies that include the lupus anticoagulant, or moderate-high titer anticardiolipin, or anti-beta 2Glycoprotein I antibodies. New research shows that APS has an estimated prevalence of about 50/100,000 population, and can occur both in patients with underlying autoimmune disease, infections, malignancies, drugs (secondary APS) and in patients without any concomitant clinical condition (primary APS).

This Special Issue is open for basic research, and will also cover original articles as well as reviews on the following topics:

Antiphospholipid antibodies: pathophysiology and molecular mechanisms;
Clinical features of antiphospholipid syndrome;
Traditional and innovative therapeutic approaches for antiphospholipid syndrome.

Prof. Dr. Cristina Belizna
Prof. Dr. Ljudmila Stojanovich
Dr. Jaume Alijotas-Reig
Guest Editor

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Published Papers (5 papers)

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Research

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15 pages, 2545 KiB

Open AccessArticle

Differences in Antiphospholipid Antibody Profile between Patients with Obstetric and Thrombotic Antiphospholipid Syndrome

by Ariadna Anunciación-Llunell, Cándido Muñoz, Dirk Roggenbuck, Stefano Frasca, Josep Pardos-Gea, Enrique Esteve-Valverde, Jaume Alijotas-Reig and Francesc Miró-Mur

Int. J. Mol. Sci. 2022, 23(21), 12819; https://doi.org/10.3390/ijms232112819 - 24 Oct 2022

Cited by 9 | Viewed by 2396

Abstract

Antiphospholipid syndrome (APS) is a systemic autoimmune condition characterised by the presence of antiphospholipid antibodies (aPL) associated with vascular thrombosis and/or pregnancy complications. In a cohort of 74 yet diagnosed APS individuals fulfilling Sydney laboratory criteria (twice positive for lupus anticoagulant, anticardiolipin, aCL, and/or anti-β2glycoprotein I, aβ2GPI), 33 out of 74 were obstetric APS (OAPS) and 41 thrombotic APS (TAPS) patients. 39% of TAPS patients were women. Although aPL detection was persistent, we observed an oscillatory aPL positivity in 56.7% and a transient seroconversion in 32.4% of APS patients at enrolment. Thus, we tested their sera in a line immunoassay that simultaneously detected IgG or IgM for criteria (aCL and aβ2GPI) and non-criteria (anti-phosphatidylserine, aPS; anti-phosphatidic acid, aPA; anti-phosphatidylinositol, aPI; anti-annexin 5, aA5; anti-prothrombin, aPT; anti-phosphatidylethanolamine; anti-phosphatidylglycerol, and anti-phosphatidylcholine) aPL. OAPS and TAPS patients displayed different but overlapping clusters based on their aPL reactivities. Specifically, while OAPS patients showed higher aPA, aPS, aA5, aβ2GPI and aPT IgM levels than TAPS patients, the latter displayed higher reactivity in aCL, aPI and aA5 IgG. Eventually, with a cut-off of the 99^th percentile established from a population of 79 healthy donors, TAPS patients significantly tested more positive for aCL and aA5 IgG than OAPS patients, who tested more positive for aPA, aPS and aβ2GPI IgM. Transiently seronegative APS patients showed non-criteria aPL positivity twice in sera obtained 3 months apart. Overall, our data show that APS patients presented clusters of aPL that define different profiles between OAPS and TAPS, and persistent non-criteria aPL positivity was observed in those who are transiently seronegative. Full article

(This article belongs to the Special Issue Antiphospholipid Syndrome)

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11 pages, 835 KiB

Open AccessArticle

Asymmetric Dimethylarginine Is a Marker of Endothelial Dysfunction in Thrombotic Antiphospholipid Syndrome Patients

by Natasa Stanisavljevic, Ljudmila Stojanovich, Aleksandra Djokovic, Brankica Todic, Violeta Dopsaj, Jovica Saponjski, Dusan Saponjski, Olivera Markovic, Cristina Belizna, Marija Zdravkovic and Dragomir Marisavljevic

Int. J. Mol. Sci. 2022, 23(20), 12309; https://doi.org/10.3390/ijms232012309 - 14 Oct 2022

Cited by 3 | Viewed by 1587

Abstract

Objective: The potential contribution of asymmetric dimethylarginine (ADMA) and high-sensitivity C reactive protein (hsCRP) to endothelial dysfunction in APS patients has not been studied in detail, until now. The study involved 105 APS patients (59 diagnosed with primary APS (PAPS) and 46 APS associated with systemic lupus erythematosus (SAPS)) who were compared to 40 controls. Endothelial dysfunction was assessed by measurement of flow-mediated dilatation (FMD) and glyceryl trinitrate dilatation (NMD) of the brachial artery. ADMA (micromol/L) was analyzed by ELISA. Results: FMD in patients with APS was significantly lower than that of the controls (p < 0.001), with no difference between the PAPS and the SAPS groups. ADMA and hsCRP concentrations were significantly higher in the patient cohort than in the control group (p < 0.001, p = 0.006, respectively), as was the case with the SAPS group as compared to the PAPS group (p < 0.001, p = 0.022, respectively). FMD impairment correlated to ADMA (ρ 0.472, p < 0.001) and to hsCRP (ρ 0.181, p = 0.033). In the regression model, the ADMA concentration confirmed the strength of its association (B 0.518, SE 0.183, Wald 8.041, p = 0.005, Exp(B) 1.679, 95% CI 1.174–2.402) to FMD impairment. The synergistic probability model of ADMA and hsCRP caused FMD impairment when the positivity of β2GPIIgG was added. ADMA may be used as a simple and low-cost tool for verifying the presence of endothelial dysfunction in APS patients. According to the results of the study, we could presume that hsCRP, together with aPL, has a preparatory effect on the endothelium in causing endothelial dysfunction. Full article

(This article belongs to the Special Issue Antiphospholipid Syndrome)

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12 pages, 1913 KiB

Open AccessCommunication

IgA Antiphospholipid Antibodies in Antiphospholipid Syndrome and Systemic Lupus Erythematosus

by Tatiana Reshetnyak, Fariza Cheldieva, Maria Cherkasova, Alexander Lila and Evgeny Nasonov

Int. J. Mol. Sci. 2022, 23(16), 9432; https://doi.org/10.3390/ijms23169432 - 21 Aug 2022

Cited by 9 | Viewed by 2157

Abstract

Objective: To define the role of IgA antibodies to cardiolipin (aCL) and IgA antibodies to beta-2 glycoprotein 1 (anti-β₂-GP1) in the development of vascular complications in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Material and methods: A total of 187 patients with one of the following diagnoses: primary APS (PAPS), probable APS, SLE with APS, and SLE without APS. The comparison group consisted of 49 patients with other rheumatic diseases (RD), the control group included 100 relatively healthy individuals (without RD, oncological pathology, and infectious diseases). All patients underwent standard clinical, laboratory, and instrumental examinations before being included in the study and during follow-up. The aPL study included the determination of IgG/IgM aCL, IgG/IgM anti-β₂-GP1 by enzyme-linked immunosorbent assay (ELISA), IgG/IgM/IgA aCL, IgG/IgM/IgA anti-β₂-GP1 by chemiluminescence analysis (CLA), and lupus anticoagulant (LA). Results: IgA aCL were detected in 75 (40%) of the 187 patients with APS and SLE, in none of the comparison group, and in 2 (2%) of the control one. IgA anti-β₂-GP1 were detected in 63 (34%) of the 187 patients with APS and SLE, in none of the patients in the comparison group, and in one (1%) of the control group. The prevalence of IgA aCL and IgA anti-β₂-GP1 and their levels were statistically significantly higher in patients with APS (PAPS and SLE + APS) than the levels in patients with SLE and those of the comparison and control groups (p < 0.05). IgA aCL and IgA anti-β₂-GP1 were significantly associated with thrombosis in APS (χ² = 4.96; p = 0.02 and χ² = 4.37; p = 0.04, respectively). The risk of thrombosis was 2.04 times higher in patients with positive IgA aCL than in patients without these antibodies, as well as in patients with positive IgA anti-β₂-GP1; it was twice as high as in patients without antibodies. There was a high specificity of IgA aCL and IgA anti-β₂-GP1 for both the diagnosis of APS and its clinical manifestations, despite a low sensitivity. Conclusions: The study revealed a relationship of thrombosis and APS with IgA aCL and IgA anti-β₂-GP1. There was a high specificity of IgA aCL and IgA anti-β₂-GP1 (95% and 93%, respectively) for the diagnosis of APS with a low sensitivity (54% and 44%, respectively). There were no patients with isolated positivity of IgA aCL and IgA anti-β₂-GP1. Full article

(This article belongs to the Special Issue Antiphospholipid Syndrome)

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14 pages, 962 KiB

Open AccessArticle

Determination of Thrombogenicity Levels of Various Antiphospholipid Antibodies by a Modified Thrombin Generation Assay in Patients with Suspected Antiphospholipid Syndrome

by Pavla Bradáčová, Luděk Slavík, Adéla Skoumalová, Jana Úlehlová, Eva Kriegová, Gayane Manukyan, David Friedecký, Barbora Piskláková, Jana Ullrychová, Jana Procházková and Antonín Hluší

Int. J. Mol. Sci. 2022, 23(16), 8973; https://doi.org/10.3390/ijms23168973 - 11 Aug 2022

Cited by 3 | Viewed by 2757

Abstract

Antiphospholipid syndrome (APS) is a hypercoagulable state accompanied by the presence of heterogeneous antiphospholipid antibodies (aPL), which nonspecifically affect hemostasis by the presence of lupus anticoagulans (LA), anticardiolipin antibodies (aCL), antibodies against β2-glycoprotein-I (anti-β2GPI), but also non-criteria antibodies such as antibodies against β2-glycoprotein-I domain I (anti-DI), anti-phosphatidylserine/prothrombin (anti-PS/PT), anti-annexin V, and many others. The main target of the antibodies is the activated protein C (APC) system, the elimination of which can manifest itself as a thrombotic complication. The aim of this study was to determine the thrombogenicity of antibodies using a modified protein C-activated thrombin generation assay (TGA) on a group of 175 samples suspected of APS. TGA was measured with/without APC and the ratio of both measurements was evaluated (as for APC resistance), where a cut-off was calculated ≤4.5 (90th percentile) using 21 patients with heterozygous factor V Leiden mutation (FV Leiden heterozygous). Our study demonstrates the well-known fact that multiple positivity of different aPLs is a more severe risk for thrombosis than single positivity. Of the single antibody positivity, LA antibodies are the most serious (p value < 0.01), followed by aCL and their subgroup anti-DI (p value < 0.05). Non-criteria antibodies anti-annexin V and anti-PT/PS has a similar frequency occurrence of thrombogenicity as LA antibodies but without statistical significance or anti-β2GPI1 positivity. The modified TGA test can help us identify patients in all groups who are also at risk for recurrent thrombotic and pregnancy complications; thus, long-term prophylactic treatment is appropriate. For this reason, it is proving increasingly beneficial to include the determination antibodies in combination with modified TGA test. Full article

(This article belongs to the Special Issue Antiphospholipid Syndrome)

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Review

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11 pages, 1090 KiB

Open AccessReview

Hydroxychloroquine as an Immunomodulatory and Antithrombotic Treatment in Antiphospholipid Syndrome

by Deepa J. Arachchillage, Mike Laffan and Charis Pericleous

Int. J. Mol. Sci. 2023, 24(2), 1331; https://doi.org/10.3390/ijms24021331 - 10 Jan 2023

Cited by 10 | Viewed by 7024

Abstract

Antiphospholipid syndrome (APS) is an acquired highly prothrombotic disorder in which thrombo-inflammatory antiphospholipid antibodies (aPL) cause thrombosis via multiple mechanisms, including endothelial damage and activation. Obstetric complications in APS are caused by placental thrombosis, inflammation and complement activation. Anticoagulation is poorly effective in some patients especially those with triple positive aPL who are at ~30% risk of thrombosis recurrence within 10 years. Increasing therapeutic anticoagulation intensity may be beneficial but leads to excess bleeding with serious complications, such as intracerebral haemorrhage. Nonetheless, anticoagulation is still the mainstay of treatment despite the autoimmune nature of APS. The antimalarial immunomodulatory drug hydroxychloroquine (HCQ) has been used for many years for the treatment of inflammatory rheumatic diseases. HCQ has complex pleiotropic mechanisms of action upon multiple cell types. The proposed biological processes that HCQ regulates support the hypothesis that it may be a successful adjunctive treatment in the prevention of recurrent thrombosis and pregnancy complications. Full article

(This article belongs to the Special Issue Antiphospholipid Syndrome)

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