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Antiphospholipid Syndrome: Clinical Advances in Diagnosis and Management
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Antiphospholipid Syndrome: Clinical Advances in Diagnosis and Management

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Immunology".

Deadline for manuscript submissions: 15 September 2025 | Viewed by 3480

Special Issue Editors

Dr. Mattia Galli

E-Mail Website
Guest Editor
Maria Cecilia Hospital, GVM Care & Research, Cotignola, Italy
Interests: cardiology; thrombosis; coagulation; antiplatelet therapy; anticoagulant therapy
Dr. Alessandra Ida Celia

E-Mail Website
Guest Editor
Rheumatology Unit, Department of Clinical Internal, Anesthetic and Cardiovascular Sciences, Sapienza University of Rome, Rome, Italy
Interests: antiphospholipid syndrome; antiphospholipid antibodies; systemic lupus erythematosus; immunosuppressive therapy; diagnosis; management

Special Issue Information

Dear Colleagues,

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the presence of antiphospholipid antibodies, which predispose individuals to arterial and venous thrombosis as well as pregnancy complications. This syndrome can occur either in isolation (primary APS) or in conjunction with other autoimmune diseases, most notably systemic lupus erythematosus (secondary APS).

Recent clinical advances have revolutionized both the diagnosis and management of antiphospholipid syndrome (APS). However, these remain a challenge for clinicians.

This Special Issue is dedicated to the critical contributions of hematologists, rheumatologists, obstetricians, and other specialists for comprehensive management and improving long-term outcomes in patients with APS. The aim of this Special Issue, which will include original papers and reviews, is to provide an up-to-date overview of advances and progress in the treatment of APS, which will aid in the accurate diagnosis and management of this devastating disease to prevent its harmful consequences.

Dr. Mattia Galli
Dr. Alessandra Ida Celia
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • antiphospholipid syndrome
  • antiphospholipid antibodies
  • systemic lupus erythematosus
  • immunosuppressive therapy
  • diagnosis
  • management

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Published Papers (4 papers)

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Research

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15 pages, 955 KiB  
Article
Pregnancy Outcomes in Non-Criteria Obstetric Antiphospholipid Syndrome: Analysis of a Cohort of 91 Patients
by Sara Beça, Núria Baños, Maria Borrell, Estíbaliz Ruiz-Ortiz, Albert Pérez-Isidro, Ricard Cervera, Joan Carles Reverter, Dolores Tàssies and Gerard Espinosa
J. Clin. Med. 2024, 13(24), 7862; https://doi.org/10.3390/jcm13247862 - 23 Dec 2024
Viewed by 1275
Abstract
Background: The clinical and laboratory features of patients with non-criteria obstetric antiphospholipid syndrome (NC-OAPS), as well as their pregnancy outcomes and ideal treatment are not clearly determined. The aim of this study is to describe the characteristics and outcomes of pregnancies in [...] Read more.
Background: The clinical and laboratory features of patients with non-criteria obstetric antiphospholipid syndrome (NC-OAPS), as well as their pregnancy outcomes and ideal treatment are not clearly determined. The aim of this study is to describe the characteristics and outcomes of pregnancies in NC-OAPS and compare them with an obstetric APS (OAPS) cohort. Methods: This is a retrospective study conducted on a cohort of women referred to a high-risk obstetric unit of a tertiary hospital. Women that were classified as having OAPS or NC-OAPS were included and compared in terms of clinical and laboratory characteristics, management, and subsequent pregnancy outcomes. Results: We identified 107 women with 143 pregnancies, 91 with NC-OAPS and 16 with OAPS. There were no differences in demographic features between both groups. Women with NC-OAPS were more likely to have recurrent implantation failure and were predominantly positive for a single antiphospholipid antibody (aPL) subtype. Both groups were treated similarly (low dose aspirin plus low molecular weight heparin in 87.4% of NC-OAPS and 83.3% of OAPS, p > 0.05). Live birth rate (82.4% and 75.0%, respectively, p > 0.05) and adverse pregnancy outcomes (31.6% vs. 37.5%, p > 0.05) in subsequent pregnancies during follow-up were also similar between groups. Conclusions: This study revealed differences in the previous pregnancy morbidity and aPL profiles in women with NC-OAPS and OAPS, although the therapeutic approach and the outcomes of subsequent pregnancies were similar in both groups. Full article
(This article belongs to the Special Issue Antiphospholipid Syndrome: Clinical Advances in Diagnosis and Management)
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Review

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17 pages, 2140 KiB  
Review
Perspective on Renal Involvement in Antiphospholipid Syndrome: Implications for Diagnosis, Pathogenesis, and Treatment
by Ariela Hoxha, Dorella Del Prete, Irene Condonato, Francesca K. Martino, Marco Lovisotto, Federico Nalesso and Paolo Simioni
J. Clin. Med. 2025, 14(10), 3326; https://doi.org/10.3390/jcm14103326 - 10 May 2025
Viewed by 354
Abstract
Antiphospholipid syndrome (APS) can affect the kidneys, leading to renal artery and vein thrombosis, allograft loss following transplantation, and microvascular damage referred to as aPL-nephropathy (aPL-N). APL-N is a complex and frequently underdiagnosed condition characterized by an incomplete understanding of its etiopathogenesis and [...] Read more.
Antiphospholipid syndrome (APS) can affect the kidneys, leading to renal artery and vein thrombosis, allograft loss following transplantation, and microvascular damage referred to as aPL-nephropathy (aPL-N). APL-N is a complex and frequently underdiagnosed condition characterized by an incomplete understanding of its etiopathogenesis and associated with unfavorable renal outcomes. The 2023 ACR/EULAR classification criteria for APS included aPL-N within the microvascular domain. The gold standard for aPL-N is the biopsy, revealing lesions associated with acute thrombotic microangiopathy and chronic vascular changes. Nevertheless, reluctance for biopsies due to anticoagulation and thrombocytopenia underscores the need for noninvasive diagnostics. Common clinical features include hypertension, microscopic hematuria, proteinuria, and renal insufficiency. Antiphospholipid antibodies seem crucial to kidney damage through thrombotic and inflammatory processes. Studies and experimental models of thrombotic microangiopathy lesions suggest the involvement of the complement cascade, tissue factor, and mammalian target of the rapamycin complex activation pathway. Currently, the management of aPL-N is based mainly on expert opinion, with limited evidence supporting the use of anticoagulants, leading to controversy in their application. Treatment may include heparin, intravenous immunoglobulin, plasma exchange, and targeted therapies tailored to aPL-N mechanisms. Future multicenter studies are essential to clarify their roles. The goal of this review is to inform clinicians and create a research agenda to address the unmet needs in diagnosing and managing APL-N. Full article
(This article belongs to the Special Issue Antiphospholipid Syndrome: Clinical Advances in Diagnosis and Management)
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12 pages, 1071 KiB  
Review
Update on the Laboratory Diagnosis of Lupus Anticoagulant: Current Challenges and Clinical Involvement
by Ana Marco-Rico
J. Clin. Med. 2025, 14(8), 2791; https://doi.org/10.3390/jcm14082791 - 18 Apr 2025
Viewed by 469
Abstract
Lupus anticoagulant (LAC) is a heterogeneous mix of autoimmune antibodies that prolongs phospholipid-dependent clotting assays. Its diagnosis can be a real challenge in the hemostasis laboratory. In this review, the author describes the main pitfalls affecting the preanalytical phase and how to proceed [...] Read more.
Lupus anticoagulant (LAC) is a heterogeneous mix of autoimmune antibodies that prolongs phospholipid-dependent clotting assays. Its diagnosis can be a real challenge in the hemostasis laboratory. In this review, the author describes the main pitfalls affecting the preanalytical phase and how to proceed to reduce interferences. Because of the heterogeneity of these autoantibodies, two assays with different mechanism of action should be performed to detect the majority of LACs. The dilute Russell’s viper venom test and the use of a reagent very sensitive to LAC derived from the activated partial thromboplastin time, using silica as the activator, are the most frequent techniques. The algorithms for LAC detection are reported here, and every laboratory is encouraged to introduce its own diagnostic procedure. Results should be expressed in ratio to reduce inter- and intravariability. In addition, the effect of anticoagulation in LAC assays and possible strategies for a correct diagnosis are provided. Full article
(This article belongs to the Special Issue Antiphospholipid Syndrome: Clinical Advances in Diagnosis and Management)
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19 pages, 5686 KiB  
Review
Non-Criteria Obstetric Antiphospholipid Syndrome: Myth or Reality?
by Sara Beça, Maria Borrell, Ricard Cervera, Francesc Figueras, Alfons Nadal, Gerard Espinosa and Núria Baños
J. Clin. Med. 2025, 14(4), 1299; https://doi.org/10.3390/jcm14041299 - 15 Feb 2025
Viewed by 971
Abstract
Women with adverse pregnancy outcomes suggestive of obstetric antiphospholipid syndrome (OAPS), but not fulfilling clinical and/or laboratory international classification criteria, are increasingly recognized both in clinical practice and in the literature. This entity is termed non-criteria OAPS (NC-OAPS). It includes clinical scenarios such [...] Read more.
Women with adverse pregnancy outcomes suggestive of obstetric antiphospholipid syndrome (OAPS), but not fulfilling clinical and/or laboratory international classification criteria, are increasingly recognized both in clinical practice and in the literature. This entity is termed non-criteria OAPS (NC-OAPS). It includes clinical scenarios such as two unexplained pregnancy losses, three non-consecutive pregnancy losses, late pre-eclampsia/eclampsia/signs of placental insufficiency, or recurrent implantation failure, as well as positive low-titers of antiphospholipid antibodies (aPLs) and non-classical aPLs. To address the NC-OAPS heterogeneity, a nomenclature proposal was developed. In recent years, retrospective and prospective cohort studies have been designed to clarify the characteristics and outcomes of the different subsets of NC-OAPS. In general, the studies support that NC-OAPS may benefit from treatment with antithrombotic, anticoagulant and/or immunomodulator agents, but several considerations must be made on the robustness and nuances of the scientific evidence. The objective of this review is to critically analyze the available evidence supporting the diagnosis of NC-OAPS, categorize its subsets, and evaluate the impact of treatment strategies on its outcome. We also remark on questions that are still unanswered, such as the lack of consensus on diagnostic criteria or treatment protocols. Full article
(This article belongs to the Special Issue Antiphospholipid Syndrome: Clinical Advances in Diagnosis and Management)
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