Assessment and comparison of the 2023 ACR/EULAR APS criteria with the revised Sapporo criteria.

OBJECTIVE: To analyze antiphospholipid antibody (aPL)-positive patients using the 2023 American College of Rheumatology/The European Alliance of Associations for Rheumatology (ACR/EULAR) antiphospholipid syndrome (APS) classification criteria and compare the revised Sapporo criteria and the 2023 ACR/EULAR criteria and evaluate whether the 2023 ACR/EULAR criteria provide added value over the revised Sapporo criteria. METHODS: In this descriptive study, 94 aPL-positive patients (with or without APS diagnosis) were identified from two hospital-based registries (Gazi and Hacettepe University). Patients were classified into four groups to compare both criteria sets. These four groups are as follows: (1) patients classified with only the revised Sapporo criteria; (2) patients classified with only the 2023 ACR/EULAR APS criteria; (3) patients classified with both two criteria sets; and (4) patients classified with neither two criteria set. RESULTS: Of the 94 patients, 11 were classified with only the revised Sapporo criteria; one with only the 2023 ACR/EULAR APS criteria; 52 with both criteria sets; and 30 with neither set of criteria. For these 94 patients, the operating characteristics of the 2023 ACR/EULAR APS criteria, using the revised Sapporo criteria as the gold standard, the 2023 ACR/EULAR APS entry criteria demonstrated 100% sensitivity, and the 2023 ACR/EULAR APS classification criteria demonstrated 98% specificity and 82.5% sensitivity. CONCLUSION: The study emphasizes the importance of recognizing differences in clinical manifestations, such as early pregnancy loss without severe preeclampsia (PEC) and/or severe placental insufficiency (PI) and calls for a nuanced discussion on anticardiolipin (aCL) and anti-beta 2-glycoprotein-I (anti-ß2GPI) immunoglobulin G (IgG) cutoff values.

Assessment of the 2023 ACR/EULAR antiphospholipid syndrome classification criteria in a Chinese cohort: Impact on clinical practice.

OBJECTIVES: To evaluate the effectiveness of the 2023 ACR/EULAR criteria for antiphospholipid syndrome (APS) in a Chinese cohort, and compare them with the Sapporo and revised Sapporo criteria. METHODS: A cohort comprising 436 patients diagnosed with APS and 514 control subjects was enrolled, including 83 with seronegative APS and 86 classified as antiphospholipid antibody (aPL) carriers. We assessed IgG and IgM anticardiolipin antibodies (aCL) and anti-ß2-glycoprotein I (aß2GPI) antibodies using ELISA, along with a systematic collection of lupus anticoagulant data. Subsequently, we compared the sensitivity and specificity across the three classification criteria. RESULTS: The 2023 ACR/EULAR criteria exhibited improved specificity at 98 %, surpassing the revised Sapporo (90 %) and original Sapporo (91 %) criteria. However, this came with decreased sensitivity at 82 %, in contrast to higher sensitivities in the revised Sapporo (98 %) and Sapporo (91 %) criteria. Examining individual components sheds light on the scoring system's rationale within the new criteria. The inclusion of microvascular thrombosis, cardiac valve disease, and thrombocytopenia improved the identification of nine patients previously classified as "probable APS". Insufficient scoring in 78 previously diagnosed APS individuals was linked to traditional risk factor evaluations for thrombotic events, the emphasis on determining whether obstetric events are linked to severe preeclampsia (PEC) or placental insufficiency (PI), and the lower scores assigned to IgM aCL and/or aß2GPI antibody. Seronegative APS remained a challenge, as non-criteria aPL and other methods were not included. CONCLUSIONS: The new criteria presented notable advancements in specificity. This study provides detailed insights into the strengths and possible challenges of the 2023 ACR/EULAR criteria, enhancing our understanding of their impact on clinical practice.

Thrombin Generation Assay in Antiphospholipid Antibodies Positive Subjects as a Personalized Thrombotic Risk Assessment: State of the Art and Perspectives.

PURPOSE OF THE REVIEW: Thrombotic risk assessment in antiphospholipid positive (aPL +) subjects is a major challenge, and the study of in vitro thrombin generation (thrombin generation assays (TGA)) could provide useful information. Activated protein C (APC) sensitivity is involved in thrombotic events in antiphospholipid syndrome patients. We summarized methods used to assess APC sensitivity with TGA and evaluated the prognostic role of APC resistance through literature search. RECENT FINDINGS: APC resistance induced by aPL is a complex pathway. Several cross-sectional studies assessed APC sensitivity to understand thrombotic event mechanisms in aPL + subjects. Only one prospective cohort had investigated the prognostic impact of APC resistance in aPL + subjects, with a positive and significant correlation between APC sensitivity and the risk of thrombosis during the follow up (hazard ratio, 6.07 [95% CI, 1.69-21.87]). APC resistance assessed with TGA could be associated with thrombotic events in aPL + subjects.

Adjusted Global Antiphospholipid Syndrome Score (aGAPSS) and PLT, APTT: Predictive Value of Thrombotic Risk Assessment in SLE Patients.

BACKGROUND: Risk assessment of vascular thrombosis in SLE patients with the presence of antiphospholipid antibodies (aPL) remains a challenge. The adjusted global antiphospholipid syndrome score (aGAPSS) has been validated and used to predict aPL-related thrombosis in SLE patients in some countries. Relevant data of aGAPSS in thrombotic evaluation in SLE population from China has not been reported. We aim to validate aGAPSS in thrombosis assessment in Chinese patients with SLE and to explore the correlations of aGAPSS with routine laboratory parameters and their clinical significance as well. METHODS: A total of 166 consecutive SLE patients were retrospectively analyzed. Multivariate logistic regression analysis was performed to examine the impact of multiple cardiovascular risk factors and laboratory parameters in recurrent thrombosis risk in SLE. ROC was conducted to explore the discriminative ability of aGAPSS and platelet (PLT), activated partial thromboplastin time (APTT), alone or in combination. RESULTS: Significantly higher value of aGAPSS was seen in SLE patients with vascular thrombosis. ROC curve indicated that aGAPSS of 3.5 or more had the best diagnostic accuracy for the prediction of aPL-related thrombosis in SLE patients. PLT with cutoff of 187.5 x 109/L and APTT with 37.5 seconds were predictors of aPL-related thrombosis as well. The combination of aGAPSS with PLT and APTT improved AUC compared to aGAPSS alone. CONCLUSIONS: The aGAPSS could predict the risk of aPL-related vascular thrombosis in SLE patients from China. The combination of aGAPSS with PLT and APTT was first time proved to have better predictive performance in thrombosis risk assessment in SLE.

Cardiovascular risk assessment in patients with antiphospholipid syndrome: a cross-sectional performance analysis of nine clinical risk prediction tools.

OBJECTIVES: This study aimed to assess the performance of cardiovascular risk (CVR) prediction models reported by European Alliance of Associations for Rheumatology and European Society of Cardiology recommendations to identify high-atherosclerotic CVR (ASCVR) patients with antiphospholipid syndrome (APS). METHODS: Six models predicting the risk of a first cardiovascular disease event (first-CVD) (Systematic Coronary Risk Evaluation (SCORE); modified-SCORE; Framingham risk score; Pooled Cohorts Risk Equation; Prospective Cardiovascular Münster calculator; Globorisk), three risk prediction models for patients with a history of prior arterial events (recurrent-CVD) (adjusted Global APS Score (aGAPSS); aGAPSSCVD; Secondary Manifestations of Arterial Disease (SMART)) and carotid/femoral artery vascular ultrasound (VUS) were used to assess ASCVR in 121 APS patients (mean age: 45.8±11.8 years; women: 68.6%). We cross-sectionally examined the calibration, discrimination and classification accuracy of all prediction models to identify high ASCVR due to VUS-detected atherosclerotic plaques, and risk reclassification of patients classified as non high-risk according to first-CVD/recurrent-CVD tools to actual high risk based on VUS. RESULTS: Spiegelhalter's z-test p values 0.47-0.57, area under the receiver-operating characteristics curve (AUROC) 0.56-0.75 and Matthews correlation coefficient (MCC) 0.01-0.35 indicated moderate calibration, poor-to-acceptable discrimination and negligible-to-moderate classification accuracy, respectively, for all risk models. Among recurrent-CVD tools, SMART and aGAPSSCVD (for non-triple antiphospholipid antibody-positive patients) performed better (z/AUROC/MCC: 0.47/0.64/0.29 and 0.52/0.69/0.29, respectively) than aGAPSS. VUS reclassified 34.2%-47.9% and 40.5%-52.6% of patients classified as non-high-ASCVR by first-CVD and recurrent-CVD prediction models, respectively. In patients aged 40-54 years, >40% VUS-guided reclassification was observed for first-CVD risk tools and >50% for recurrent-CVD prediction models. CONCLUSION: Clinical CVR prediction tools underestimate actual high ASCVR in APS. VUS may help to improve CVR assessment and optimal risk factor management.

Assessment of antiphospholipid antibodies profiles based on severity of COVID-19 pneumonia.

Introduction: thrombotic events are the most severe complications of the coronavirus disease 2019 (COVID-19). It is known that anti-phospholipid antibodies (APL) could be involved in thrombosis mechanism. Thus, APL profiles were studied particularly in patients with severe and critical COVID-19, and their clinical impact. Methods: a retrospective study of 54 COVID-19 hospitalized patients (34 in intensive care unit (ICU) and 20 in non-ICU) was conducted. These COVID-19 patients were tested for the presence of LAC (lupus anticoagulant) using the ACLTOP750®, anti-cardiolipine (ACL) and anti-ß2glycoprotéine I (anti-ß2GPI) IgG/IgM/IgA by enzyme-linked immunosorbent assay (ELISA). IgA isotype was tested in only 25 patients. Results: anti-phospholipid antibodies were present in 74.1% of tested patients. LAC positivity was the highest (60.8%) among all patients, followed by IgM aCL (18.5%) and IgM anti-ß2GPI (14.8%). Besides, LAC and anti-ß2GPI IgA were the most predominant APL regarding the 25 patients tested for IgA isotype (52% and 24% respectively). Nine patients had thrombotic events, among them 6 were positive in APL and 5 were positive in LAC. However, there was any significant association between APL positivity or titers and thrombosis. There was also no significant difference between the two COVID-19 groups regarding APL profiles. Conclusion: given the relatively high frequency of APL and especially LAC, and given the multitude of thrombotic risk factors in these severely and critically ill COVID-19 patients, a prophylactic anticoagulation remains essential.

Mammalian Target of Rapamycin Pathway Assessment in Antiphospholipid Antibody-Positive Patients with Livedo.

OBJECTIVE: In antiphospholipid antibody (aPL) nephropathy, activation of the mammalian target of rapamycin (mTOR) contributes to endothelial cell proliferation, a key finding of aPL microvascular disease. Here, we examined mTOR activation in the skin of aPL-positive patients with livedo. METHODS: Three patient groups with livedo were studied: (1) persistently aPL-positive with systemic lupus erythematosus (SLE); (2) persistently aPL-positive without SLE; and (3) aPL-negative SLE (control). After collecting aPL-related medical history, two 5-mm skin biopsies of livedo were performed on each patient: (1) peripheral (erythematous-violaceous lesion); and (2) central (nonviolaceous area). We stained specimens for phosphorylated protein kinase B (p-AKT) and phosphorylated S6 ribosomal protein (p-S6RP) as mTOR activity markers, CD31 to identify endothelial cells, and Ki-67 to show cellular proliferation. We counted cells in the epidermis and compared mTOR-positive cell counts between peripheral and central samples, and between patient groups, using Freidman test and Wilcoxon signed-rank test. RESULTS: Ten patients with livedo reticularis were enrolled: 4 aPL-positive without SLE (antiphospholipid syndrome [APS] classification met, n = 3), 4 aPL-positive SLE (APS classification met, n = 3), and 2 aPL-negative SLE (control). In all aPL-positive patients, epidermal p-AKT and p-S6RP staining were significantly increased in both peripheral and central skin samples when compared to aPL-negative SLE controls; both were more pronounced in the lower basal layers of epidermis. CONCLUSION: Our study demonstrates increased mTOR activity in livedoid lesions of aPL-positive patients with or without SLE compared to aPL-negative patients with SLE, with more prominent activity in the lower basal layers of the epidermis. These findings may serve as a basis for further investigating the mTOR pathway in aPL-positive patients.

A multi-laboratory assessment of lupus anticoagulant assays performed on the ACL TOP 50 family for harmonized testing in a large laboratory network.

INTRODUCTION: Lupus anticoagulant (LA) testing is commonly performed within hemostasis laboratories, and the ACL TOP 50 family of instruments represent a new "single platform" of hemostasis instrumentation. Our aim was to evaluate these instruments and manufacturer reagents or alternatives for utility in LA testing. METHODS: Comparative evaluations of LA testing using newly installed ACL TOPs 550 and 750 as well as comparative assessments with existing "reference," predominantly Stago, instrumentation, and reagents. Evaluations comprised both dilute Russell viper venom time (dRVVT) and activated partial thromboplastin time (APTT)-based assays. Establishment of normal reference ranges (NRR). RESULTS: The HemosIL dRVVT-based assays showed good comparability with the existing Stago reference method (R > 0.9) and could be considered as verified as fit for purpose. A variety of APTT assays was additionally evaluated for LA utility, and we identified from the assessment good utility of a non-Werfen solution in Hyphen BioMed Cephen reagents. NRR were established based on ≥120 normal individual plasma samples. CONCLUSION: This evaluation of LA reagents on ACL TOP 50 Family instruments identified overall acceptable performance of both dRVVT (Werfen solution) and APTT (non-Werfen solution) to enable harmonization of LA testing in our large network.

Pediatric antiphospholipid syndrome: clinical features and therapeutic interventions in a single center retrospective case series.

BACKGROUND/PURPOSE: Pediatric antiphospholipid syndrome (APS) is a thromboinflammatory disease characterized by the presence of circulating antiphospholipid antibodies and either thrombotic events or pregnancy morbidity. The objective of this study was to review a large institution's experience to better understand the characteristics of children with APS. METHODS: We conducted a retrospective review of pediatric APS at a tertiary referral center. The electronic medical record system was queried from 2000 through 2019, and 21 cases were included based on meeting the revised Sapporo Classification criteria by age 18 or younger. Comparisons between primary and secondary APS patients were made with two-tailed t-tests. RESULTS: Twenty-one patients were included with a median age at diagnosis of 16 years and median follow-up of 5.8 years. Secondary APS was slightly more common than primary APS (11 vs. 10 cases) and was primarily diagnosed in the context of systemic lupus erythematosus. Two thirds of patients (67%) also had "non-criteria" manifestations of APS including thrombocytopenia, autoimmune hemolytic anemia, and livedo reticularis/racemosa. Almost half of patients (43%) had recurrent thrombosis, typically when patients were subtherapeutic or non-adherent with anticoagulation. Damage Index in Patients with Thrombotic APS (DIAPS) scores indicated a chronic burden of disease in both primary and secondary APS patients. CONCLUSION: This case series of pediatric APS provides important context regarding disease phenotypes displayed by children with APS. High prevalence of non-criteria clinical manifestations highlights the need to consider these characteristics when developing pediatric-specific classification criteria and when considering this relatively rare diagnosis in pediatric practice.

Cost-effectiveness analysis of treatments for the first episode of catastrophic antiphospholipid syndrome: A study based on the catastrophic antiphospholipid syndrome registry.

BACKGROUND: Treatments for catastrophic antiphospholipid syndrome (CAPS) rose from recommendations and consensus of international experts based on case series or case reports. We aimed to evaluate the treatment scheme with the best cost-effectiveness ratio associated with lower mortality as a high-impact clinical benefit. METHODS: The CAPS Registry was used as our source of structured data on the different therapeutic strategies, their frequency, and their effectiveness (survival). Starting from around 50 different schemes, we identified those with a mortality of less than 33% within the 18 most frequently utilized. After applying the efficiency frontier method, we included two schemes to conduct a cost-effectiveness analysis from the Colombian healthcare sector perspective. Scheme 1 (Glucocorticoids + Anticoagulation + Anti-aggregation + Intravenous IgG immunoglobulin) and scheme 2 (Glucocorticoids + Anticoagulation + Anti-aggregation + Plasma exchange) were compared in terms of costs and survival. Deterministic and probabilistic sensitivity analyses (Monte Carlo simulation) were conducted to evaluate model robustness and uncertainty. RESULTS: Our analysis uses the information corresponding to 427 cases from the CAPS registry, the majority being women (68.8%), with a mean age of 45.7 years and bearing general mortality of 38.17% (female: 38.4%, male: 37.5%). Scheme 2 was the cost-effective strategy over scheme 1. The results were robust on discrete sensitivity analysis and probability sensitivity analysis (Monte Carlo simulation). CONCLUSION: To our knowledge, this is the first economic evaluation focused on the treatment of CAPS. For the Colombian health system, schemes 1 and 2 have similar behavior; nevertheless, scheme 2 represents the best cost-effectiveness ratio. This treatment approach is highly susceptible to the allocation of resources by the system and beneficial in terms of health outcomes.

Assessment of Anti-ß2 glycoprotein1 antibody in Systemic Lupus Erythrematosus Patients.

Secondary anti-phospolipid syndrome (APS) is diagnosed in many patients with systemic lupus erythrematosus (SLE) especially with thromboembolic events and/or pregnancy loss in the presence of persistent laboratory evidence for anti-phospholipid antibody (aPL). In this work, we aimed to detect the prevalence of IgG and IgA anti-ß2 glycoprotein1 (ß2GP1) in SLE patients. Serum samples were collected from 50 female patients with SLE (25 had APS and 25 patients who did not have APS), in addition to 22 apparently healthy females with matched age as a control group. All samples from patients and control were tested for lupus anticoagulant (LA), IgG and IgA isotypes of antiß2GPI, Anti-nuclear antibody (ANA), Anti-double strand antibody (Anti-dsDNA). Number of patients positive for Anti-ß2GP1 antibodies were significantly increased in APS patients compared to non-APS patients (P=0.015). Anti-ß2GP1 IgA isotype was significantly higher in APS patients than in non-APS patients (P=0.011) and significantly correlated with deep venous thrombosis and pregnancy morbidity (P=0.004). There was no difference in anti-ß2GPI IgG isotype between APS patients and non-APS patients. We concluded that although anti-ß2GPI IgA is not within Sapparo diagnostic criteria, it seems to contribute to the pathogenesis of thrombotic manifestations of SLE and may represents a useful indicator particularly when standard aPL tests are negative.

Thrombotic risk assessment in patients with systemic lupus erythematosus: Validation of the adjusted-Global Antiphospholipid Syndrome Score (aGAPSS) in Thai patients.

BACKGROUND: The adjusted-Global Antiphospholipid Syndrome Score (aGAPSS) has been validated and used to predict antiphospholipid antibodies (aPL) related to vascular thrombosis (VT). OBJECTIVE: To validate aGAPSS for predicted aPL-related VT and pregnancy complications (PC) in Thai systemic lupus erythematosus (SLE) patients. METHODS: A cross-sectional study was performed among Thai SLE patients with clinical manifestations; history of VT and PC, cardiovascular risk factors, and aPL profiles were collected. The aGAPSS was calculated from the sum of the risk factors (hyperlipidemia = 3.0, arterial hypertension = 1.0, anti-cardiolipin antibody = 5.0, anti-b2 glycoprotein I antibody = 4.0, and lupus anticoagulant = 4.0). RESULTS: Of 132 SLE patients, 12 (9.1%) had VT and 5 (4.1%) had PC. When comparing the aGAPSS (median; interquartile range [IQR]) of patients with events (VT and/or PC) (6.5; IQR 3.3-9.0), VT (8.0; IQR 4.0-9.0), arterial thrombosis (3.5; IQR 1.0-5.8), and PC (9.0; IQR 8.0-11.5), and the aGAPSS of patients without an event (3.0; IQR 0-4.0), aGAPSS of patients with events was significantly higher, except in patients with arterial thrombosis. An aGAPSS of 4.5 or more was associated with risk of aPL-related VT (sensitivity 71.4%, specificity 76.7%), and an aGAPSS of 6.0 or more was associated with risk of aPL-PC (sensitivity 100%, specificity 84.0%). CONCLUSION: The aGAPSS could predict the risk of aPL-PC and aPL-related VT in Thai SLE patients.

The impact of various entities of antiphospholipid antibodies positivity on adverse pregnancy outcome. An epidemiological perspective.

The aim of the study was to evaluate the rate of obstetric complications and the burden of obstetric outcomes in antiphospholipid syndrome (APS), non-criteria APS and asymptomatic antiphospholipid antibodies (aPL) carriers. From 2013-2018, 163 pregnant subjects with aPL antibodies and 785 controls were enrolled. Penalized logistic regression was used to compare obstetric complications. Cases included 62 complete APS (38 %), 48 non-criteria APS (29.4 %) and 53 (32.5 %) asymptomatic aPL-carriers. Connective tissue diseases (CTDs) were diagnosed in 31.3 % of cases. The rate of high-risk aPL profile was higher (p < .01) in APS (67.7 %) compared to non-criteria (14.6 %) and aPL-carriers (9.4 %). Double/triple positivity was 33.9 % (p < .05 compared to non-criteria and aPL-carriers) in APS, 10.4 % in non-criteria and 9.4 % in aPL-carriers. The rate of adverse pregnancy outcomes were 5.6 % in controls, 41.9 % (adj.OR = 6.95 %CI = 2.7-13.5) in APS, 25 % (adj.OR = 4.4,95 %CI = 2-9.4) in non-criteria and 28.3 % (OR = 4.95 %CI = 1.8-8.8) in aPL-carriers. CTDs were independently associated with an increased risk of adverse obstetric outcomes (OR = 2.8,95 %CI = 1.36-5.89). The attributable fraction (AF) of adverse obstetric events was higher among low-risk antibodies compared to high-risk (AF = 0.27,95 %CI = 0.22-0.31 vs AF = 0.16,95 %CI = 0.16-0.2,p < .01) and among single positivity compared to double/triple positivity (AF = 0.32,95 %CI = 0.26-0.37 vs AF = 0.11,95 %CI = 0.09-0.13,p < .01) suggesting that low-risk subjects are responsible for a high burden of obstetric complications.

The importance of pregnancy planning in lupus pregnancies.

OBJECTIVE: In seeking new approaches to improve lupus pregnancy outcomes, we study the association between pregnancy planning, behaviors recommended by American College of Rheumatology's Reproductive Health Guideline 2020, and pregnancy and infant outcomes. METHODS: Lupus pregnancies in a prospective registry (1/1/2018 to 4/1/2020) were classified as planned or not-planned using the patient-reported London Measure of Unplanned Pregnancy. These groups were compared for demographics, pre-pregnancy disease activity, pregnancy planning behaviors, and delivery outcomes. RESULTS: Among 43 women with 43 singleton pregnancies the average age was 29.4 years and 42% were Black. Overall, 60% were planned pregnancies and 40% were not-planned (16 ambivalent, 1 unplanned). Women with not-planned pregnancies had lower age, income, and education, and more required Medicaid. Women with not-planned pregnancies were more likely to conceive when lupus activity was higher (p = 0.001), less likely to receive pre-pregnancy counseling with a rheumatologist (p = 0.02), and less likely to continue pregnancy-compatible medications (p = 0.03). Severe PROMISSE adverse pregnancy outcomes (APOs) and severe neonatal outcomes were higher among women with not-planned than planned pregnancies (43% vs 0% p = 0.003; 70% vs 30% p = 0.06). CONCLUSION: This study identifies pregnancy intention as a potentially modifiable risk factor for poor outcomes in women with lupus. It highlights a unique population of women with lupus at high risk for pregnancy and infant complications: those ambivalent about pregnancy. These women may not be effectively engaging in health behaviors that prevent pregnancy nor those that will prepare for a safe pregnancy. With effective pregnancy planning and contraception guidance, we may decrease their risk for maternal-fetal morbidity and mortality.

Prevalence, outcome and management of patients with SLE and secondary antiphospholipid antibody syndrome after aPL seroconversion.

OBJECTIVE: The withdrawal of oral anticoagulation (OAC) in patients with SLE and secondary aPL syndrome (SAPS) who become seronegative has not been clearly investigated to date. Our aim was to evaluate the prevalence of aPL seroconversion and the prognosis of SLE patients with SAPS who withdrew OAC after aPL negativization. METHODS: We retrospectively analysed data of all SLE patients (ACR criteria) with SAPS (Sydney criteria) prospectively followed-up in our clinic. aPL seroconversion was defined as negativization of lupus anticoagulant, aCL, and anti-ß2glycoprotein-1 antibodies on two or more consecutive measurements, at least 12 weeks apart. OAC discontinuation was defined as the definitive withdrawal of all anticoagulants. RESULTS: Fifty-five out of 513 (10.7%) SLE patients had vascular SAPS. Sixteen patients (29.1%) became aPL seronegative during follow-up. Immunosuppressive therapy predicted aPL negativization (odds ratio 5.211, 95%CI 1.341, 20.243), whereas APS diagnosis prior to that of SLE (odds ratio 0.078, 95%CI 0.008, 0.799) and triple-positive profile (odds ratio 0.264, 95%CI 0.115, 0.609) were negative predictors of aPL negativization. OAC was discontinued in 13/55 patients (23.6%), after a median follow-up of 45 months (range 1-276) from aPL seroconversion. SLE-related modifiable risk factors for thrombosis were observed in 10/13 patients (77%) at the time of the thrombotic event. No thrombotic recurrences were observed during a mean follow-up time of 44 (19) months from OAC discontinuation. CONCLUSIONS: Our results suggest that OAC can be safely discontinued in SLE patients who became persistently seronegative for aPL, at least when aPL-related thrombotic events occurred in presence of other thrombotic risk factors.

[Prospective assessment of a multidisciplinary meeting dedicated to inflammatory and vascular diseases during pregnancy]. / Évaluation prospective d'une réunion de concertation pluridisciplinaire dédiée aux pathologies inflammatoires et vasculaires lors de la grossesse : la RCP « PREGNANT ¼ au CHU de Bordeaux.

OBJECTIVES: The pluridisciplinary meeting "PREGNANT - Pregnancy and Auto-immunity, Nephropathy, Thrombophilic Disorders" at the university hospital of Bordeaux is dedicated to inflammatory and thrombophilic disorders during pregnancy. The objective of our study was to evaluate the quality of this meeting in terms of: compliance with the mandatory criteria, adequacy with standard care, homogeneity of care, becoming of proposals issued. METHODS: We conducted a prospective observational study including patients whose files were submitted to the meeting from January 2018 to June 2019. RESULTS: In all, 16 meeting were conducted with 152 cases presented. Sixty-two patients were pregnant and 90 were in preconception. The most common reasons for presentation were vasculo-placentary diseases (22.3%), systemic lupus (16.4%), venous thromboembolic diseases (15.1%) and chronic intervillositis of unknown etiology (9.8%). Other reasons were antiphospholipid antibody syndrome and repeated spontaneous miscarriages. The mandatory criteria for multidisciplinary meeting were met. For 89 cases (58.5%), the problem was dictated by recommendations. Decisions made were consistent with recommendations in 89.8% of cases. Among the 63 cases without any published recommendations (41.5%), there was some homogeneity of the proposals. In all, 92.8% of the proposals issued by the meeting were implemented. CONCLUSIONS: Multidisciplinary meeting "PREGNANT" has a prominent locoregional role in the management of patients with autoimmune, inflammatory or thrombophilic disorders in a pregnancy context.