Antiphospholipid antibody-related clinical manifestations during childhood versus adulthood: descriptive results from the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository.

Objectives: There is a limited number of studies comparing paediatric to adult antiphospholipid syndrome (APS) patients. Our objective was to analyse the characteristics of patients presenting with antiphospholipid antibody (aPL)-related clinical manifestations during childhood versus adulthood.

Methods: We retrieved baseline characteristics from an international registry of persistently aPL-positive adult patients.

Epigenetic regulation of thrombo-inflammation in Behçet and antiphospholipid syndrome.

Background: An epigenetic regulation of thrombo-inflammation has been reported in Behçet syndrome (BS), likely driven by a unique profile of three plasmatic circulating microRNAs (ci-miRNAs) (miR-206, miR-224-5p, and miR-653-5p). We compared this ci-miRNAs expression in BS and antiphospholipid syndrome (APS), the prototype of acquired pro-thrombotic autoimmune disease. To further corroborate the hypothesis that shared mechanisms drive the thrombotic process in BS and APS, we further assessed their thrombin generation assay (TGA) profile.

Reasons for Breastfeeding Avoidance: A Multicenter Insight in Mothers With Systemic Lupus Erythematosus.

Background: Limited research exists on breastfeeding among women with systemic lupus erythematosus, despite known benefits of human milk.

Research Aim: To investigate reasons for breastfeeding avoidance among postpartum women with systemic lupus erythematosus.

Methods: This is a descriptive study, conducted in January 2023, combining retrospective pregnancy outcome data collection with a structured telephone questionnaire.

JAK inhibitors for the management of rheumatic diseases when antiphospholipid syndrome co-exists: case-based safety considerations.

JAK inhibitors (JAKi) are small molecules that interact with JAK proteins, modulating the JAK-STAT signaling pathway, which plays a significant, though not yet fully understood, role in immune regulation. Due to the breadth of their mechanism of action, JAKi have shown promising results in the treatment of various immune-mediated diseases across different fields such as rheumatology or dermatology, and may represent a valuable therapeutic option for patients with multiple coexisting immune-mediated conditions. However, recent years have seen growing concerns regarding their use due to an observed increase in cardiovascular and thromboembolic events compared to anti-TNF drugs, which may complicate administration in patients with additional associated risk factors.

Multicenter study to improve clinical interpretation of anticardiolipin and anti-β2-glycoprotein I antibody test results for diagnosis of antiphospholipid syndrome.

Background: Anticardiolipin (aCL) and anti-β2-glycoprotein I (aβGPI) antibodies are laboratory markers important for antiphospholipid syndrome (APS) diagnosis and classification. There is an important interassay variation among aCL and aβGPI assays.

Objectives: This study aimed to harmonize aCL and aβGPI test result interpretation across assays.

Thrombocytopenia and autoimmune hemolytic anemia in antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid syndrome alliance for clinical trials and InternatiOnal networking (APS ACTION) clinical database and repository ("Registry").

Background/PurposeAPS ACTION Registry was created to study the natural course of antiphospholipid syndrome (APS) over 10 years in persistently antiphospholipid antibody (aPL) positive patients with or without systemic autoimmune rheumatic diseases (SARDs). Our primary objective was to compare the characteristics of aPL-positive patients with or without thrombocytopenia (TP) and/or autoimmune hemolytic anemia (AIHA).MethodsThe registry inclusion criteria are positive aPL based on the Revised Sapporo APS Classification Criteria, tested at least twice within 1 year prior to enrollment.

Problem Solved? An Individual Ratio between Point-of-Care and Venous International Normalized Ratio Values in Two Patients with Antiphospholipid Syndrome: Two Case Reports.

Antiphospholipid syndrome (APS) is a rare autoimmune disorder characterized by thromboembolic and obstetric complications in the presence of persistent antiphospholipid antibodies (aPL). Treatment aims to prevent recurrent thrombosis, primarily using anticoagulation therapy with vitamin K antagonists (VKA). Monitoring of VKA therapy relies on the International Normalized Ratio (INR), which can be assessed using point-of-care testing (POCT).

Antiphospholipid IgG Certified Reference Material ERM-DA477/IFCC: a tool for aPL harmonization?

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 the laboratory diagnosis of antiphospholipid syndrome (APS). We evaluated CRM's ability to represent the heterogeneity of APS patient anti-β2GPI antibodies and to calibrate IgG anti-β2GPI methods.

Methods: We characterized CRM for its reactivity against domain-1, using the QUANTA Flash β2GPI-domain-1 assay, and against domains-4-5 of β2GPI, and single-domain-deleted β2GPI molecules using in-house ELISAs.

Diagnostic, research, and real-life effect of the 2023 EULAR-ACR classification criteria for antiphospholipid syndrome.

The role of classification criteria is particularly important in rheumatic diseases compared with other medical disorders, as the complexity and overlapping symptoms of these conditions make diagnosis challenging. Moreover, the absence of established diagnostic criteria further complicates diagnosing patients. Classification criteria can assist health-care professionals and patients as a diagnostic aid.

Predictors of Mortality in Antiphospholipid Antibody-Positive Patients: Prospective Results From Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking Clinical Database and Repository.

Objective: The objective was to determine the mortality rate as well as the causes and predictors of death in antiphospholipid antibody (aPL)-positive patients with and without antiphospholipid syndrome (APS) classification.

Methods: The inclusion criterion for the multicenter international Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) registry is positive aPLs according to the Revised Sapporo Classification Criteria tested within one year before enrollment. Patients are observed every 12 ± 3 months with clinical data and blood collection.

The Interconnection Between Systemic Lupus Erythematosus and Diet: Unmet Needs, Available Evidence, and Guidance-A Patient-Driven, Multistep-Approach Study.

Background/objectives: Inflammation and immunological dysregulation are central to systemic lupus erythematosus (SLE), a complex autoimmune disease. Recently, there has been increasing interest in the potential role of dietary factors in SLE. This study aimed to explore the relationship between diet and SLE by addressing patient needs, conducting a systematic review, and providing guidance to the patient community.

How to treat patients with thrombotic antiphospholipid syndrome in 2024?

Antiphospholipid syndrome (APS) encompasses a range of clinical conditions, particularly thrombotic or obstetrical manifestations, associated with the presence of antiphospholipid antibodies. Managing thrombotic APS in daily clinical practice can be challenging and requires thorough risk stratification and tailored treatment strategies. Primary prophylaxis focuses on correcting the traditional thrombotic risk factors and, in certain situations, may include low‑dose aspirin and / or prophylactic anticoagulants (eg, low‑molecular‑weight heparin).

Assessing the steroid-sparing effect of biological agents in randomized controlled trials for lupus: a scoping review.

Prompt disease control of flares in patients with systemic lupus erythematosus (SLE) is a priority in treatment strategy planning. However, the long-term dosage-related collateral effects of glucocorticoids (GCs) have pushed researchers towards the identification and utilization of novel biological agents that could both induce and maintain low disease activity and remission, especially in the context of lupus nephritis (LN). This scoping review aims at assessing the current evidence of the potential steroid-sparing effect of biologic therapies by reviewing phase II and phase III randomized, placebo-controlled trials involving SLE/LN patients.

2023 ACR/EULAR classification criteria in existing research cohorts: an international study.

Objective: To assess the impact of the updated ACR/EULAR APS classification criteria on two large research cohorts.

Methods: Consecutive patients who tested persistently positive for at least one aPL in the last three years were enrolled. The first APS Sydney index event was considered and computed for the comparison between Sydney and 2023 APS criteria.

Deciphering the clinical significance of longitudinal antiphospholipid antibody titers.

In antiphospholipid syndrome (APS), the risk of clinical manifestations increases with higher titers of antiphospholipid antibodies (aPL). Despite the adoption of aPL titers in the classification approach to aPL-positive subjects, the value of longitudinal monitoring of those titers in the follow-up is still debated, being well studied only in systemic lupus erythematosus (SLE). The literature suggests that the rate of aPL positivity decreases during follow-up in primary APS, estimating that seroconversion occurs in between 8.

The estimated prevalence of antiphospholipid antibodies and criteria-antiphospholipid syndrome in subjects with renal thrombotic microangiopathy.

Background: While the prevalence of antiphospholipid antibodies (aPL) in venous and arterial thrombotic events had already been estimated by previous studies, the prevalence of aPL in subjects with Thrombotic Microangiopathy (TMA) is still not fully elucidated. Thus, we conducted a systematic review to estimate the frequency of aPL in subjects with biopsy-proven renal TMA.

Methods: We conducted in the PubMed database a search for English-language studies investigating the presence of aPL in subjects with biopsy-proven renal TMA from January 1985 to December 2022.

16th International congress on antiphospholipid antibodies task force report on antiphospholipid syndrome laboratory diagnostics and trends.

Classification criteria for antiphospholipid syndrome (APS) require IgG or IgM isotypes of the anticardiolipin (aCL) antibodies, anti-β2 glycoprotein I (anti-β2GPI) antibodies, and/or the lupus anticoagulant (LA) to satisfy the laboratory disease definition. Over the past 20 years, non-criteria antiphospholipid antibodies (aPL) directed to other proteins of the coagulation cascade (i.e.