Primary antiphospholipid syndrome in pediatrics: New criteria, new opportunities.

Abstract

ObjectiveWe present the clinical, laboratory, and treatment characteristics of patients diagnosed with primary antiphospholipid syndrome according to the 2023 ACR/EULAR APS classification criteria.Material and methodsA retrospective study was conducted in patients under 18 years of age, diagnosed with primary antiphospholipid syndrome according to the 2023 ACR/EULAR classification criteria, at the General Hospital of the National Medical Center La Raza, Mexico, from January 2013 to January 2025. Data on clinical manifestations, laboratory, and treatment were collected.ResultsWe present data from 40 patients, 22 female and 18 male, with a mean age at diagnosis of 12.8 years. The time to diagnosis was 15.4 weeks. Thrombosis occurred in 14 patients. Thrombocytopenia in 34. Autoimmune hemolytic anemia in 18, Fisher Evans syndrome in 17. Livedo in 33, skin ulcers in 2, and Raynaud's phenomenon in 15. Epileptic seizures in 2 and chorea in 1. We did not find cardiac valvular involvement. Renal involvement was found in 4. Prolonged aPTT in 39 (97.5%), positive lupus anticoagulant in 39, positive anticardiolipin IgG in 32, positive anticardiolipin IgM in 18, anti-B2GPI IgG performed in 17 patients, positive in 15, anti-B2GPI IgM performed in 12, positive in 4. Treatment consisted of anticoagulation in 13 patients, antiplatelet agents in 29, steroids in 35 immunosuppressants in 33, and rituximab in 6. One patient died from an alveolar hemorrhage.ConclusionsThe 2023 ACR/EULAR APS criteria improve the classification of pediatric patients by including non-thrombotic criteria; however, there are other non-thrombotic manifestations that are common in children that should be taken into account in this population.