Insights into the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria: findings from a cohort of 205 patients with primary APS.

Objectives: The 2023 ACR/EULAR antiphospholipid syndrome (APS) classification criteria distinguish between anticardiolipin (aCL) or anti-β2-glycoprotein I (aβ2GPI) IgG vs IgM isotypes, having isolate IgM positivity a low weight, insufficient for APS classification, and define aCL and aβ2GPI thresholds based on fixed cut-off values. We aimed to assess the performance of the 2023 ACR/EULAR criteria in a cohort of primary vascular APS patients (PAPS), previously classified according to the Sydney criteria. Additionally, we evaluated the risk of thrombotic recurrence in patients re-classified or not according to the new criteria.

Antiphospholipid antibody carriers and patients with quiescent antiphospholipid syndrome show persistent subclinical complement activation.

Objectives: Complement activation has been advocated as one mechanism by which aPLs can induce thrombosis. In patients with catastrophic APS or re-thrombosis, enhanced complement activation has been shown, even in the quiescent phase of the disease. We aimed to assess complement activation and to investigate its association with clinical variables in aPL-positive patients with a favourable disease course.

Prevalence and adverse consequences of delayed diagnosis and misdiagnosis in thrombotic antiphospholipid syndrome. An observational cohort study and a review of the literature.

Obiectives: This study aims to prospectively evaluate the frequency and adverse consequences of diagnostic delay and misdiagnosis in a cohort of patients with thrombotic antiphospholipid syndrome (TAPS). In addition, a systematic review of the literature concerning the diagnostic delay and misdiagnosis of TAPS was carried out.

Methods: Patient enrollment occurred between 1999 and 2022.

Short and long-term outcomes of children with autoimmune congenital heart block treated with a combined maternal-neonatal therapy. A comparison study.

Objective: The short and long-term outcomes of children with anti-Ro/La-related congenital heart block treated with a combined maternal-neonatal therapy protocol were compared with those of controls treated with other therapies.

Study Design: Sixteen mothers were treated during pregnancy with a therapy consisting of daily oral fluorinated steroids, weekly plasma exchange and fortnightly intravenous immunoglobulins and their neonates with intravenous immunoglobulins (study group); 19 mothers were treated with fluorinated steroids alone or associated to intravenous immunoglobulins or plasma exchange (control group).

Result: The combined-therapy children showed a significantly lower progression rate from 2nd to 3rd degree block at birth, a significant increase in heart rate at birth and a significantly lower number of pacemaker implants during post-natal follow-up with respect to those treated with the other therapies.