Silvia Rosina1, Cecilia Beatrice Chighizola2, Angelo Ravelli1,3,4, Rolando Cimaz5,6,7. 1. Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 2. Experimental Laboratory of Immunological and Rheumatologic Researches, Immunology and Rheumatology Unit, San Luca Hospital, IRCCS Istituto Auxologico Italiano, Via Zucchi 18, Cusano Milanino, 20095, Milan, Italy. c.chighizola@auxologico.it. 3. University of Genoa, Genoa, Italy. 4. Sechenov First Moscow State Medical University, Moscow, Russian Federation. 5. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. 6. RECAP_RD, University of Milan, Milan, Italy. 7. Pediatric Rheumatology Unit, ASST G. Pini & CTO, Milan, Italy.
Abstract
PURPOSE OF REVIEW: Elucidating the pathogenic mechanisms mediated by antiphospholipid antibodies (aPL) might exert important clinical implications in pediatric antiphospholipid syndrome (APS). RECENT FINDINGS: aPL are traditionally regarded as the main pathogenic players in APS, inducing thrombosis via the interaction with fluid-phase and cellular components of coagulation. Recent APS research has focused on the role of β2 glycoprotein I, which bridges innate immunity and coagulation. In pediatric populations, aPL should be screened in appropriate clinical settings, such as thrombosis, multiple-organ dysfunction, or concomitant systemic autoimmune diseases. Children positive for aPL tests often present non-thrombotic non-criteria manifestations or asymptomatic aPL positivity. In utero aPL exposure has been suggested to result in developmental disabilities, warranting long-term follow-up. The knowledge of the multifaceted nature of pediatric APS should be implemented to reduce the risk of underdiagnosing/undertreating this condition. Hopefully, recent pathogenic insights will open new windows of opportunity in the management of pediatric APS.
PURPOSE OF REVIEW: Elucidating the pathogenic mechanisms mediated by antiphospholipid antibodies (aPL) might exert important clinical implications in pediatric antiphospholipid syndrome (APS). RECENT FINDINGS: aPL are traditionally regarded as the main pathogenic players in APS, inducing thrombosis via the interaction with fluid-phase and cellular components of coagulation. Recent APS research has focused on the role of β2 glycoprotein I, which bridges innate immunity and coagulation. In pediatric populations, aPL should be screened in appropriate clinical settings, such as thrombosis, multiple-organ dysfunction, or concomitant systemic autoimmune diseases. Children positive for aPL tests often present non-thrombotic non-criteria manifestations or asymptomatic aPL positivity. In utero aPL exposure has been suggested to result in developmental disabilities, warranting long-term follow-up. The knowledge of the multifaceted nature of pediatric APS should be implemented to reduce the risk of underdiagnosing/undertreating this condition. Hopefully, recent pathogenic insights will open new windows of opportunity in the management of pediatric APS.
Authors: R Cervera; J Font; J A Gómez-Puerta; G Espinosa; M Cucho; S Bucciarelli; M Ramos-Casals; M Ingelmo; J-C Piette; Y Shoenfeld; R A Asherson Journal: Ann Rheum Dis Date: 2005-02-11 Impact factor: 19.103
Authors: Paolo Durigutto; Claudia Grossi; Maria Orietta Borghi; Paolo Macor; Francesca Pregnolato; Elena Raschi; Michael P Myers; Philip G de Groot; Pier Luigi Meroni; Francesco Tedesco Journal: Haematologica Date: 2018-11-15 Impact factor: 9.941