Assessment of triple antiphospholipid antibody-positive patients based on clinical and laboratory domains of 2023 ACR/EULAR antiphospholipid syndrome classification criteria.
Academic Article
Overview
abstract
OBJECTIVES: Triple aPL-positive patients (positive for lupus anticoagulant [LA], anticardiolipin antibody [aCL], and anti-β2-glycoprotein-I antibody [aβ2GPI]) are at higher risk for severe clinical manifestations compared to double or single aPL-positive patients. This study aimed to evaluate the characteristics of triple aPL-positive patients using the 2023 ACR/EULAR Antiphospholipid Syndrome (APS) Classification Criteria (CC) and compare those with different aCL/aβ2GPI isotypes. METHODS: We retrospectively analyzed an APS-specialized practice cohort (Feb 2016-May 2024). Triple aPL positivity was defined as persistently positive LA, aCL IgG/M ≥ 40 ELISA Units, and aβ2GPI IgG/M ≥ 40 ELISA Units. Demographic, clinical, and serologic features were assessed, and patients with aCL/aβ2GPI IgG (±IgM) were compared to those with IgM-only. RESULTS: Among 751 patients, 193 had confirmed persistent triple aPL positivity (165 IgG [±IgM], 28 IgM-only). Clinical domain involvement included macrovascular venous thromboembolism (46 %), macrovascular arterial thrombosis (39 %), microvascular (35 %), obstetric (46 %), cardiac valve (16 %), and hematologic (34 %). Patients with aCL/aβ2GPI IgG (±IgM) had significantly higher 2023 ACR/EULAR CC total clinical domain score, greater involvement of macrovascular and microvascular domains, and higher use of long-term anticoagulation, steroids, and immunosuppression compared to IgM-only patients. Notably, almost half of IgM-only patients, who were older, lacked clinical domain involvement. CONCLUSIONS: Our findings highlight distinct clinical profiles in triple aPL-positive patients based on aCL/aβ2GPI isotypes. While IgG (±IgM) isotypes were associated with higher clinical domain scores and severe manifestations, the role of IgM-only isotypes warrants further mechanistic and clinical investigation even in triple aPL profile.
