Development of clinical manifestations in individuals positive for antiphospholipid antibodies according to the 2023 ACR/EULAR serological domains.

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Development of clinical manifestations in individuals positive for antiphospholipid antibodies according to the 2023 ACR/EULAR serological domains.

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Clinical manifestations, management, and outcomes in patients with triple positive antiphospholipid antibodies
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Clinical manifestations, management, and outcomes in patients with triple positive antiphospholipid antibodies

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  • Cite Count Icon 743
  • 10.1111/j.1365-2141.2012.09037.x
Guidelines on the investigation and management of antiphospholipid syndrome
  • Feb 8, 2012
  • British Journal of Haematology
  • David Keeling + 4 more

This guidance updates and replaces the previous guideline on the investigation and management of antiphospholipid syndrome (APS) published in 2000 (Greaves et al, 2000), though where there have not been changes we refer back to them when appropriate. The guidance is updated with reference to relevant publications since 2000. Publications known to the writing group were supplemented with additional papers identified by searching PubMed for publications in the last 11 years using the key words: lupus anticoagulant, anticardiolipin, antiphospholipid, b2–glycoprotein I, antiprothrombin and limits (clinical trial, randomized control trial, meta-analysis, humans, core clinical journals, English language). The writing group produced the draft guideline, which was subsequently revised by consensus by members of the Haemostasis and Thrombosis Task Force of the British Committee for Standards in Haematology. The guideline was then reviewed by a sounding board of approximately 50 UK haematologists, the Royal College of Obstetricians and Gynaecologists (RCOG), and the British Committee for Standards in Haematology (BCSH) Committee and comments incorporated where appropriate. The ‘GRADE’ system was used to quote levels and grades of evidence, details of which can be found at http://www.bcshguidelines.com/BCSH_PROCESS/EVIDENCE_LEVELS_AND_GRADES_OF_RECOMMEN DATION/43_GRADE.html. The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of patients with antiphospholipid syndrome though individual patient circumstances may dictate an alternative approach.

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Concordance between Acr/EULAR and Sapporo Criteria for Antiphospholipid Syndrome: New Domains Unveil Associations with Procoagulant Platelets
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Concordance between Acr/EULAR and Sapporo Criteria for Antiphospholipid Syndrome: New Domains Unveil Associations with Procoagulant Platelets

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Laboratory criteria for antiphospholipid syndrome: communication from the SSC of the ISTH
  • Apr 1, 2018
  • Journal of Thrombosis and Haemostasis
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Laboratory criteria for antiphospholipid syndrome: communication from the SSC of the ISTH

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  • 10.1182/blood-2019-131438
Incidence, Natural History and Outcomes of Transient and Persistent Antiphospholipid Antibodies in Children and Young Adults with Provoked Venous Thromboembolism: Analysis of the Kids-DOTT Trial
  • Nov 13, 2019
  • Blood
  • Marisol Betensky + 10 more

Incidence, Natural History and Outcomes of Transient and Persistent Antiphospholipid Antibodies in Children and Young Adults with Provoked Venous Thromboembolism: Analysis of the Kids-DOTT Trial

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A review on management of antiphospholipid syndrome in clinical practice
  • Sep 13, 2023
  • Italian Journal of Medicine
  • Carmine Siniscalchi + 5 more

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombotic or obstetric events occurring in patients with persistent antiphospholipid antibodies. Thrombotic APS is characterized by venous, arterial, or microvascular thrombosis. The diagnosis is accepted when both one clinical and one laboratory criteria according to the updated Sapporo classification are established. APS may occur in combination with other autoimmune diseases, mainly systemic lupus erythematosus, or in its primary form. Long-term anticoagulation with a vitamin K antagonist is the standard of care for patients who develop thrombosis, considering the high rate of recurrent thrombosis. The current international guidelines are not in favor of recommending direct oral anticoagulants for secondary prevention of thrombotic antiphospholipid syndrome, especially in the context of arterial thrombosis and triple-positive antiphospholipid patients. The most common approach, endorsed by the American College of Chest Physicians guidelines is the combination of heparin and low-dose aspirin (75-100 mg) daily for women who fulfill the clinical and serologic criteria for obstetric APS. New potential therapeutic approaches are under evaluation but actually the anticoagulation remains the cornerstone of treatment.

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The additional use of hydroxychloroquine can improve the live birth rate in pregnant women with persistent positive antiphospholipid antibodies: A systematic review and meta-analysis
  • Mar 18, 2021
  • Journal of Gynecology Obstetrics and Human Reproduction
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The additional use of hydroxychloroquine can improve the live birth rate in pregnant women with persistent positive antiphospholipid antibodies: A systematic review and meta-analysis

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  • 10.1016/j.arcped.2015.08.007
Lien entre trouble du spectre autistique de l’enfant et anticorps antiphospholipides : une étude cas–témoin
  • Sep 16, 2015
  • Archives de Pédiatrie
  • F Lageix + 11 more

Lien entre trouble du spectre autistique de l’enfant et anticorps antiphospholipides : une étude cas–témoin

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  • 10.3390/jcm14030733
Antiphospholipid Syndrome: A Comprehensive Clinical Review.
  • Jan 23, 2025
  • Journal of clinical medicine
  • Vasileios Patriarcheas + 7 more

Background: Antiphospholipid syndrome (APS) is a rare systemic autoimmune disease characterized by persistent antiphospholipid antibodies (aPL) in combination with recurrent thrombosis in the veins and/or arteries, obstetric morbidity, and various non-thrombotic associated complications. APS can be primary, as an isolated condition, or secondary in the context of another autoimmune disease, especially systemic lupus erythematosus. This comprehensive clinical review aims to summarize the current understanding of APS pathogenesis, diagnostic approaches, and treatment strategies for this unique clinical entity. Methods: A comprehensive review of the existing literature on APS was conducted, focusing on pathophysiological mechanisms, current diagnostic criteria, and therapeutic approaches. Results: APS pathogenesis involves complex interactions between aPL, phospholipid-binding proteins, and the coagulation cascade. Apart from the cardinal features of thrombosis and APS-related obstetric morbidity, APS is associated with a wide spectrum of clinical manifestations. Diagnosis remains challenging due to overlapping symptoms with other conditions, and clinicians should maintain a high index of suspicion in order to set the diagnosis. The recently published 2023 ACR/EULAR criteria although not definitive for clinical decision-making, these criteria offer clinicians a valuable tool to aid in determining whether further investigation for APS is warranted. Continued refinement of these criteria through ongoing feedback and updates is anticipated. Treatment strategies center on anticoagulation, but individualized approaches are necessary. Conclusions: Early diagnosis and multidisciplinary management of APS are critical to reducing morbidity and improving outcomes. Moreover, familiarization with the 2023 ACR/EULAR criteria is encouraged, recognizing that ongoing feedback and updates will contribute to their ongoing refinement and improvement. While VKAs remain the mainstay of treatment for most APS patients further research is needed to optimize treatment strategies and deepen our understanding of APS's underlying disease mechanisms.

  • Abstract
  • 10.1136/annrheumdis-2024-eular.897
OP0214 ASSOCIATIONS OF REMNANT CHOLESTEROL WITH ALL-CAUSE AND CAUSE-SPECIFIC THROMBOTIC RISK IN INDIVIDUALS WITH ANTIPHOSPHOLIPID SYNDROME
  • Jun 1, 2024
  • Annals of the Rheumatic Diseases
  • J Zhao + 5 more

Background:Antiphospholipid syndrome (APS) is notably linked to thrombotic events, particularly cardiovascular disease (CVD). The role of remnant cholesterol (RC) in predicting CVD risk is established, yet its relationship with thrombotic...

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POS0777 ANTIPHOSPHOLIPID RELATED LARGE VESSEL LESIONS: NOT ONLY THROMBOSIS
  • May 19, 2021
  • Annals of the Rheumatic Diseases
  • H Jiang + 3 more

POS0777 ANTIPHOSPHOLIPID RELATED LARGE VESSEL LESIONS: NOT ONLY THROMBOSIS

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The impact of hydroxychloroquine treatment on pregnancy outcome in women with antiphospholipid antibodies
  • Sep 30, 2015
  • American Journal of Obstetrics and Gynecology
  • S Sciascia + 5 more

The impact of hydroxychloroquine treatment on pregnancy outcome in women with antiphospholipid antibodies

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  • 10.3389/fimmu.2025.1639065
Novel advances on pathophysiological mechanisms, clinical manifestations, and treatment of antiphospholipid syndrome
  • Aug 21, 2025
  • Frontiers in Immunology
  • Qing-Nan Zhu + 9 more

Antiphospholipid antibody syndrome (APS) is an autoimmune disorder characterized by arterial and venous thrombosis, pregnancy-related complications, and persistent antiphospholipid antibodies. These manifestations pose significant risks to patient health and reproductive outcomes. Initially regarded as a manifestation of systemic lupus erythematosus (SLE), APS exhibits a close epidemiological association with SLE, occurring at significantly higher incidence in SLE patients. The precise pathophysiological relationship between these diseases remains unclear. Nevertheless, as an independent clinical disease, research on APS pathological mechanisms continues to advance comprehensively. The publication of the “2023 ACR/EULAR antiphospholipid syndrome classification criteria” provides refined diagnostic standards. Consequently, this review synthesizes prior studies to clarify APS pathophysiological mechanisms, explore its relationship with SLE, update emerging treatments, and provide insights for clinical management.

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