Antiphospholipid antibody-related clinical manifestations during childhood versus adulthood: descriptive results from the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository.
Academic Article
Overview
abstract
OBJECTIVES: There is a limited number of studies comparing paediatric to adult antiphospholipid syndrome (APS) patients. Our objective was to analyse the characteristics of patients presenting with antiphospholipid antibody (aPL)-related clinical manifestations during childhood versus adulthood. METHODS: We retrieved baseline characteristics from an international registry of persistently aPL-positive adult patients. Clinical events were grouped as vascular and non-vascular. We compared the frequency of and the timeline between vascular and non-vascular events for different age groups at the time of their first aPL-related manifestations: a) paediatric- (0-17 years) versus adult-onset (18-75 years); and b) based on narrower age intervals. Secondly, we analysed the timeline between the first aPL-related clinical event and first aPL positivity. RESULTS: Of 787 patients, 447 (57%) had only vascular events, 108 (14%) only non-vascular events, and 232 (29%) both. Compared to adult-onset patients (n=742), paediatric-onset patients (n=45) presented more commonly with a non-vascular event (49% vs. 19%, p=0.0001). The percentage of patients presenting with a non-vascular event mostly decreased with each increasing age group. Timeline analysis demonstrated 317 (40%) patients had a positive aPL test within the same calendar year (c-y) of the first clinical event, 207 (26%) within 1 to 3 c-y, and 263 (33%) more than 3 c-y. CONCLUSIONS: Our analysis of an international registry for persistently aPL-positive patients demonstrates that patients with paediatric-onset aPL-related manifestations more commonly present with non-vascular events. These results highlight the importance of understanding the clinical differences between paediatric and adult APS patients, which have diagnostic, therapeutic, and research implications.
