Rafael Gálvez-Sánchez

Background: The DIAPS scale is a useful tool for quantifying organ damage in antiphospholipid syndrome (APS). However, little is known about the kinetics of organ damage in antiphospholipid antibody (aPL) carriers. This study aimed to characterize damage progression in aPL carriers, assess the impact of 2023 ACR/EULAR laboratory domains, autoimmune disease, and thromboprophylaxis on this progression, and determine whether organ damage at diagnosis is associated with the later development of clinical manifestations. Methods: A retrospective observational study was conducted, including patients who met the 2023 ACR/EULAR domains 7 or 8 without clinical symptoms between January 2018 and March 2024. A total of 985 patients were analysed. Results: The overall mean DIAPS score was slightly higher in patients with persistent LA compared with those without (0.429 [IQR 0.134–0.723] vs. 0.350 [IQR 0.132–0.569]). The time*autoimmune disease interaction was significant (F = 4.532, p = 0.000), indicating a steeper increase in DIAPS among patients with associated autoimmune disease. Primary thromboprophylaxis with antiplatelet agents did not modify DIAPS progression (F = 1.357, p = 0.220). Finally, organ damage at the time of serological diagnosis was associated with a higher likelihood of progression to clinical APS. Conclusions: Among aPL carriers, persistent LA positivity emerged as the most robust serological predictor of organ damage accrual. Accumulated organ damage appears to progress more rapidly in aPL carriers with an associated autoimmune disease. Organ damage at diagnosis was associated with an increased likelihood of progressing to clinical APS.

To evaluate the clinical applicability of the 2023 ACR/EULAR serologic classification criteria for antiphospholipid syndrome (APS) in a cohort of pregnant women across the APS spectrum, and to assess the association between serologic burden and clinical manifestations, obstetric outcomes, and treatment response, a retrospective cohort study was conducted including 190 pregnant women with persistent antiphospholipid antibody (aPL) positivity. Patients were classified according to the ACR/EULAR serologic criteria into two groups: high serologic burden (≥3 points) and low serologic burden (≤2 points). Clinical, obstetric, therapeutic, and reproductive variables were compared between groups. Nearly one-third of women did not meet the 2023 ACR/EULAR serologic threshold. Although most patients with thrombotic or obstetric APS fulfilled the ACR/EULAR serologic threshold, 28% of women with obstetric APS and 35.3% of those with pregnancy-related morbidity had ≤2 serologic points. Baseline demographic characteristics were similar between groups. Obesity (23.9% vs. 10%; p = 0.04) and obstetric comorbidity (17.9% vs. 5.4%; p = 0.02) were more frequent among patients with higher serologic burden. Rates of obstetric complications were comparable between groups. The most commonly prescribed treatment was low-dose aspirin combined with low-molecular-weight heparin, with no differences according to serologic classification. Live birth rates and adverse pregnancy outcomes were comparable between groups, with favorable outcomes observed in treated pregnancies. Serologic burden as defined by the 2023 ACR/EULAR criteria does not reliably predict obstetric complications, nor identify a clinically more severe subgroup. These findings highlight that clinical judgment beyond formal classification frameworks remains essential when managing pregnant women with antiphospholipid antibodies.

Background: Behçet’s Disease (BD) was traditionally classified according to the International Study Group (ISG), where oral ulcers were mandatory. The International Team for the Revision of the International Criteria for BD (ICBD) introduced a scoring system instead. Our aim was to assess (a) sensitivity, (b) concordance between ISG and ICDB criteria in global and severe BD cases (ocular, vascular, and neurological), and (c) evaluate their clinical implications. Methods: Retrospective cohort study including 142 BD patients diagnosed in a well-defined population in Northern Spain, between January 1980 and November 2023. Both ISG and ICBD criteria were compared, sensitivity and concordance were assessed using Prevalence-Adjusted and Bias-Adjusted Kappa (PABAK) and the unadjusted Kappa. Results: A total of 142 BD patients diagnosed by expert rheumatologists (73 men; mean age of 36.4) were studied. Among them, 84 met ISG criteria, while 116 fulfilled ICBD criteria. Sensitivity of ISG and ICBD criteria in the overall cohort was (59.1% and 81.6%), respectively. Among patients with severe manifestations (ocular, vascular, or neurological), sensitivity increased to 71.2% for ISG and 92.5% for ICBD. Overall concordance was moderate (Kappa = 0.490), with 70.4% of patients classified identically. When adjusting prevalence and bias, concordance improved slightly (PABAK = 0.549). Of the 32 patients classified as BD exclusively by ICBD, 7 were receiving anti-TNF therapy, and 2 were receiving apremilast. Conclusions: The ICBD criteria demonstrated higher sensitivity than the traditional ISG criteria in classifying BD, particularly in severe cases. Classifying these additional patients under ICBD facilitated the initiation of on-label biologic treatments, potentially enhancing BD management, especially for severe cases.

Background/Objectives: Retinal vein occlusion (RVO) represents a common ophthalmological disorder that, if untreated, often leads to severely impaired vision. The classic vascular risk factors, aging and glaucoma, represent the core pathogenic factors for RVO. However, antiphospholipid syndrome (APS) has been involved in a non-negligible number of patients with RVO. The main objective of the present study was to assess the performance of the new 2023 ACR/EULAR classification criteria for APS in a cohort of patients with RVO fulfilling the Sydney classification criteria. Methods: A prospective study of consecutive patients with RVO diagnosed with APS in a third-level university hospital. The new 2023 ACR/EULAR classification criteria for APS were applied to all patients. Vascular risk factors, the antiphospholipid antibody (aPL) profile, clinical management, and clinical outcomes were assessed and compared between those fulfilling the Sydney and the 2023 ACR/EULAR criteria. Results: Sixty-nine RVO-APS patients were included in the study. After applying the new classification criteria, 18 patients (26.1%) did not fulfill the new criteria for APS. Specifically, 17 (24.6%) were excluded due to the new Domain 8 (p < 0.001) as they presented only aPL IgM serology, and 1 patient (1.4%) was excluded due to having high venous thromboembolic risk (VTE) with a clinical domain score < 3. Interestingly enough, the presence of high arterial risk (45.1% vs. 50%; p = 0.72) was greater than the presence of high VTE (3.9% vs. 5.6%; p = 0.99); in both cases, the 51 RVO-APS patients were classified with the 2023 ACR/EULAR criteria, and the 18 cases were excluded according to the new classification criteria. Except for the expected differences in serological domains (Domain 7, p < 0.001, and Domain 8, p < 0.001), we did not find other significant differences in terms of prognosis or risk of recurrence between both groups of patients. Conclusions: The implementation of the new ACR/EULAR 2023 classification criteria for APS resulted in the exclusion of about one out of four previously diagnosed RVO-APS patients. The higher prevalence of manifestations of high arterial risk compared with high VTE among both newly classified and excluded APS patients highlights the importance of monitoring cardiovascular risk factors for both the prevention and the management of potential retinal and cardiovascular events.