Navigating antiphospholipid syndrome: from personalized therapies to cutting-edge research DOI Creative Commons
Karen Kortright‐Maldonado,

Bruno Eduardo Reyes‐Torres,

Lilian Stephany Cabrera-Lopez

et al.

Rheumatology Advances in Practice, Journal Year: 2024, Volume and Issue: 9(1)

Published: Dec. 18, 2024

APS is an autoimmune disorder characterized by thrombosis and pregnancy complications, primarily driven aPLs such as LA, aCL anti-β2 glycoprotein I (a-β2GPI). Despite advances in anticoagulation therapies, managing refractory cases remains challenging. Emerging including rituximab, eculizumab HCQ, show potential addressing the underlying mechanisms of APS. Additionally, research into genetic environmental factors, particularly gut microbiome's role through molecular mimicry, suggests new therapeutic pathways. Diagnostic advancements, adjusted Global Antiphospholipid Syndrome Score (aGAPSS), metabolomic profiling MRI, have improved risk stratification early detection. Non-traditional biomarkers like anti-phosphatidylserine/prothrombin (aPS/PT) anti-Domain antibodies further enhance assessment. Future should aim to validate these approaches, optimizing patient outcomes minimizing long-term complications.

Language: Английский

Update on antiphospholipid syndrome DOI Creative Commons
Maria G. Tektonidou

Lara D. Veeken, Journal Year: 2024, Volume and Issue: 63(SI), P. SI1 - SI3

Published: Feb. 1, 2024

Language: Английский

Citations

8
Postnatal health of infants born to mothers with autoimmune diseases when treated with hydroxychloroquine DOI Creative Commons

Viviana Matys,

Francesca Rizzo, Maria Pia De Carolis

et al.

Frontiers in Lupus, Journal Year: 2025, Volume and Issue: 3

Published: Jan. 28, 2025

Introduction This retrospective cohort study aimed to observe the postnatal health of infants born mothers with systemic autoimmune rheumatic diseases treated hydroxychloroquine (HCQ) during pregnancy. Methods A total 312 pregnancies patients who suffered from different were considered. Pregnancy data collected; a telephone follow-up questionnaire was successfully completed in 182 detect long-term pediatric outcome. The women took pregnancy defined as “HCQ group” and compared did not take hydroxychloroquine, “non-HCQ group”. Results higher prevalence multiple maternal detected HCQ group, comparison that non-HCQ group ( p = 0.0015). Despite consisting more complicated conditions, obstetrical neonatal outcomes similar between two groups. Regarding health, 40% revealed no pathologies versus 25% children 0.0368). Discussion protective role on should be further evaluated prospective multicenter studies.

Language: Английский

Citations

0
Update on Stroke Prevention and Treatment in Patients with Antiphospholipid Antibody Syndrome DOI

Helena K. Xeros,

Michael Bates, Rafid Mustafa

et al.

Current Treatment Options in Neurology, Journal Year: 2025, Volume and Issue: 27(1)

Published: Jan. 29, 2025

Language: Английский

Citations

0
β2GPI-targeted polymeric nanoparticles form a protective layer to prevent vascular thrombosis in an anti-phospholipid syndrome model DOI Creative Commons
Paolo Durigutto, Maria Cristina Grimaldi, Sara Bozzer

et al.

Frontiers in Immunology, Journal Year: 2025, Volume and Issue: 16

Published: Feb. 27, 2025

Anti-phospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombotic vascular occlusion and maternal morbidity. Anti-coagulants remain pivotal drugs for the management of APS, but significant proportion patients do not benefit from long-term anti-coagulation may require an alternative therapy to prevent antibody deposition thrombosis. We have developed therapeutic approach based on use safe polymeric nanoparticles that selectively target beta2-glycoprotein I (β2GPI) deposited endothelial cells (tNPs). Their efficacy was tested in rat model APS infusing patients' sera containing medium-high titer antibodies against domain β2GPI. The tNPs bearing CH2-deleted anti-β2GPI recombinant as targeting agent recognize β2GPI failed induce blood clot formation. infused into rats immediately before competed with antibodies, preventing their binding and, consequence, resulted thrombus formations mesenteric vessels. Similar results were obtained injecting 24 hours administration Our findings suggest β2GPI-targeted represent stable formation vessel be used control thrombosis developing result acute triggering events.

Language: Английский

Citations

0
Antiphospholipid IgG Certified Reference Material ERM®-DA477/IFCC: a tool for aPL harmonization? DOI Creative Commons
C. Grossi, Liesbet Deprez, Caterina Bodio

et al.

Clinical Chemistry and Laboratory Medicine (CCLM), Journal Year: 2025, Volume and Issue: unknown

Published: March 20, 2025

Abstract Objectives The Certified Reference Material (CRM) ERM ® -DA477/IFCC is a new polyclonal IgG anti-beta2-glycoprotein I (anti-β2GPI) material for the harmonization of laboratory diagnosis antiphospholipid syndrome (APS). We evaluated CRM’s ability to represent heterogeneity APS patient anti-β2GPI antibodies and calibrate methods. Methods characterized CRM its reactivity against domain-1, using QUANTA Flash β2GPI-domain-1 assay, domains-4-5 β2GPI, single-domain-deleted β2GPI molecules in-house ELISAs. used Lite ELISA, CLIA, EliA™ FEIA methods evaluate anti-Cardiolipin (anti-CL) activity. Four (in-house FEIA) were also calibration efficacy, alongside 133 clinical samples (CSs) 99 controls. Results showed high anti-domain-1 activity no anti-domain-4-5 at recommended assay dilution. domain-dependent-β2GPI profiles comparable with full-blown APS. There was acceptable dilution linearity anti-CL assays R 2 ranging from 0.957 0.997. For four assays, led good comparability average result CSs two assays. New cut-offs calculated this work improved in quantitative results between three assays: 85 % concordance compared 66 assay-specific-calibration. Conclusions representative anti-β2GPI/CL should improve method harmonization. However, level achievable affected by differences selectivity among

Language: Английский

Citations

0
The role of chronic tonsillitis in the habitual miscarriage of pregnancy of immune origin DOI Open Access
Polina V. Filipeva, В. М. Свистушкин,

P. A. Kiryushchenkov

et al.

Aspirantskiy Vestnik Povolzhiya, Journal Year: 2025, Volume and Issue: 25(1), P. 40 - 44

Published: March 5, 2025

The prevalence of chronic tonsillitis (CT) varies from 5 to 12%. Against the background CT, a large number disorders are formed, including in reproductive system. Among problems practical obstetrics, spontaneous miscarriages account for 10 20% cases. Unexplained losses can have immunological causes those associated with CT. Approximately women recurrent pregnancy loss (RPL) elevated serum levels autoantibodies predominant predominance antiphospholipid antibodies (APLA). There is neuro-reflex mechanism influence CT on formation pathology. afferent connections palatine tonsils most important subcortical formations, particular structures posterior region hypothalamus, been studied – they first react antigens. Infectious agents play key role genesis APLA formation, since molecular mimicry. mimicry bacteria and viruses relation anti-β2HP1 main by which pathogens induce synthesis these genetically predisposed patients. an initiating immune response against It should be recognized that condition patients infertility underestimated modern clinical practice.

Language: Английский

Citations

0
Catastrophic Antiphospholipid Syndrome: A Review of Current Evidence and Future Management Practices DOI Open Access

Ayoyimika O Okunlola,

Temitope O Ajao,

Mwila Sabi

et al.

Cureus, Journal Year: 2024, Volume and Issue: unknown

Published: Sept. 19, 2024

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by blood clots and pregnancy complications due to antiphospholipid antibodies. Catastrophic APS (CAPS), a severe variant, leads multiorgan failure often fatal. Pathogenesis involves antibodies, particularly anti-beta-2-glycoprotein I (aβ2GPI), which trigger endothelial cell (EC) activation, cytokine release, prothrombotic state. Infections, surgeries, other triggers can precipitate CAPS, leading widespread microthromboses systemic inflammatory responses. CAPS predominantly affects younger patients those with lupus erythematosus (SLE), high mortality rate, though recent treatment advances have improved survival. Diagnosing identifying clinical manifestations, including rapid organ involvement small vessel occlusions, confirmed histopathology antibody levels. The registry data indicate that commonly affected organs include kidneys, lungs, central nervous system, the heart, prevalence of anticoagulant anticardiolipin antibodies (aCL). Current management strategies focus on therapeutic anticoagulation, immunosuppressive therapies like corticosteroids, adjunct treatments such as plasmapheresis intravenous immunoglobulin (IVIG). Early use glucocorticoids combination therapy has significantly outcomes. In life-threatening cases, especially microangiopathy, experts recommend performing plasma exchange (PE). Patients associated conditions or refractory cases may receive cyclophosphamide (CY) rituximab while considering PE for treatment. Maintenance anticoagulation appropriate international normalized ratio (INR) crucial prevent recurrence. This article reviews pathogenesis epidemiology CAPS. It also examines current strategies, discusses challenges controversies these strategies. hereafter offers recommendations future outlines directions further research.

Language: Английский

Citations

3
Atomistic Characterization of Beta-2-Glycoprotein I Domain V Interaction with Anionic Membranes DOI Open Access
Hale S. Hasdemir, Nicola Pozzi, Emad Tajkhorshid

et al.

bioRxiv (Cold Spring Harbor Laboratory), Journal Year: 2024, Volume and Issue: unknown

Published: March 21, 2024

Interaction of beta-2-glycoprotein I (

Language: Английский

Citations

0
Atomistic characterization of β2-glycoprotein I domain V interaction with anionic membranes DOI Creative Commons
Hale S. Hasdemir, Nicola Pozzi, Emad Tajkhorshid

et al.

Journal of Thrombosis and Haemostasis, Journal Year: 2024, Volume and Issue: 22(11), P. 3277 - 3289

Published: July 22, 2024

Interaction of β

Language: Английский

Citations

0
Kidney whole-transcriptome profiling in primary antiphospholipid syndrome reveals complement, interferons and NETs-related gene expression DOI Creative Commons
Maria G. Tektonidou, Kleio‐Maria Verrou,

Harikleia Gakiopoulou

et al.

Lara D. Veeken, Journal Year: 2024, Volume and Issue: 63(11), P. 3184 - 3190

Published: Aug. 6, 2024

Abstract Objective Pathogenesis of antiphospholipid syndrome (APS) remains poorly elucidated. We aimed to evaluate for the first time kidney transcriptome profiles in primary APS vs systemic lupus erythematosus (SLE) and control subjects. Methods performed RNA sequencing on archival formalin-fixed paraffin-embedded biopsies from (n = 4), SLE 5) 3) individuals, differential gene expression analysis (DGEA) enrichment using ontology (GO) CORUM, KEGG Reactome pathway databases. Results Two-dimensional projection showed a distinct profile kidneys samples, but similar SLE. DGEA controls returned 276 upregulated 217 downregulated genes, while comparison between identified 75 111 genes. In enriched GO terms were (innate) immune response, inflammatory leucocyte lymphocyte activation, cytokine production T cell activation. CORUM revealed complement-related genes (C3, C4A, C4B). Expression levels logFC values 2.25 (P 1.58e-05) C3, 2.17 2.69e-06) C4A 2.135 3.7e-06) C4B controls, without differences Interferon (IFN) alpha/beta signalling was by Reactome. nine IFN-regulated found (P-values ≤ 0.001 all). Examining neutrophil-extracellular traps (NETs)-related expression, 13 15 NETs-related exhibited higher not Conclusion Complement, interferon are highly expressed tissues, similarly SLE, pointing out role innate immunity nephropathy pathogenesis potential treatment targets.

Language: Английский

Citations

0