Pediatric antiphospholipid syndrome: clinical features and therapeutic interventions in a single center retrospective case series

Pediatric Rheumatology

Claire Hoy¹  Yu Zuo¹  Kelsey Gockman¹  Jason S. Knight¹  Jacqueline A. Madison²  Ajay Tambralli² 

[1] Department of Internal Medicine, Division of Rheumatology, University of Michigan, 1150 West Medical Center Drive, 48109, Ann Arbor, MI, USA;Department of Pediatrics, Division of Pediatric Rheumatology, University of Michigan, Ann Arbor, MI, USA;Department of Internal Medicine, Division of Rheumatology, University of Michigan, 1150 West Medical Center Drive, 48109, Ann Arbor, MI, USA;

关键词: Pediatrics;    Antiphospholipid syndrome;    Antiphospholipid antibodies;    Thrombosis;    Systemic lupus erythematosus;    Damage Index (DIAPS);    Non-criteria manifestations;    Anti-phosphatidylserine/prothrombin;   

DOI  :  10.1186/s12969-022-00677-8

来源: Springer

【 摘 要 】

Background/purposePediatric antiphospholipid syndrome (APS) is a thromboinflammatory disease characterized by the presence of circulating antiphospholipid antibodies and either thrombotic events or pregnancy morbidity. The objective of this study was to review a large institution’s experience to better understand the characteristics of children with APS.MethodsWe conducted a retrospective review of pediatric APS at a tertiary referral center. The electronic medical record system was queried from 2000 through 2019, and 21 cases were included based on meeting the revised Sapporo Classification criteria by age 18 or younger. Comparisons between primary and secondary APS patients were made with two-tailed t-tests.ResultsTwenty-one patients were included with a median age at diagnosis of 16 years and median follow-up of 5.8 years. Secondary APS was slightly more common than primary APS (11 vs. 10 cases) and was primarily diagnosed in the context of systemic lupus erythematosus. Two thirds of patients (67%) also had “non-criteria” manifestations of APS including thrombocytopenia, autoimmune hemolytic anemia, and livedo reticularis/racemosa. Almost half of patients (43%) had recurrent thrombosis, typically when patients were subtherapeutic or non-adherent with anticoagulation. Damage Index in Patients with Thrombotic APS (DIAPS) scores indicated a chronic burden of disease in both primary and secondary APS patients.ConclusionThis case series of pediatric APS provides important context regarding disease phenotypes displayed by children with APS. High prevalence of non-criteria clinical manifestations highlights the need to consider these characteristics when developing pediatric-specific classification criteria and when considering this relatively rare diagnosis in pediatric practice.

【 授权许可】

CC BY