Sloan Elizabeth, Wright Tracey, Zuo Yu
Division of Pediatric Rheumatology, Department of Pediatrics, UT Southwestern Medical Center, Dallas, USA.
Division of Pediatric Rheumatology, Department of Pediatrics, Texas Scottish Rite Hospital for Children, Dallas, USA.
Lupus. 2021 Apr;30(5):828-832. doi: 10.1177/09612033211002256. Epub 2021 Mar 18.
Antiphospholipid antibodies (aPL) have been extensively reported in children, but investigations into thrombotic risks associated with aPL positivity in pediatric patients is scarce. Positive aPL are not uncommon in pediatric connective tissue diseases (CTD), but identification and management of these patients is challenging due to lack of validated criteria and a paucity of data. In this study, we identify potential additional risk factors for thrombosis in a unique cohort of pediatric aPL positive carriers.
Retrospective chart review was performed on 491 pediatric patients with CTD seen in our institution from 2001 to 2019. Patients without persistently moderate to high titer aPL at least 12 weeks apart were excluded. Univariate analysis was performed to evaluate correlation between different risk factors and thrombotic events.
Seventy-one aPL positive children with underlying CTD are included in this cohort. The majority (87%) are female and of Hispanic ethnicity (56%). Mean age of the cohort at the diagnosis of connective tissue disease is 12.7 (SD 2.6) years, and mean age of first positive aPL is 13.3 (SD 2.5) years. Average length of follow-up is 4.3 (SD 2.5) years. Four (5.6%) patients experienced arterial thrombosis, and 11 (15.5%) had venous thrombosis. Fifty-seven (80.3%) patients did not have any thromboembolic events. Among traditional risk factors and signs of endothelial injury, only Raynaud's phenomena demonstrated significant association with arterial thrombosis (OR = 8.4, 95%CI 1.13-111, P = 0.039), and hypertension or anti-hypertensive use demonstrated significant association with venous thrombosis (OR = 8.387, 95%CI 1.2 - 94, P = 0.02).
Data from our cohort suggest that Raynaud's phenomenon is a potential predictor of arterial thrombosis while the presence of hypertension or anti-hypertensive medication use is a potential predictor of venous thrombosis in aPL positive pediatric carriers. Further studies investigating pediatric aPL profiles and risk factors for development of thrombosis are needed to help guide clinicians in caring for these challenging patients.
抗磷脂抗体（aPL）在儿童中已有广泛报道，但关于儿科患者aPL阳性相关血栓形成风险的研究却很少。儿科结缔组织病（CTD）中aPL阳性并不罕见，但由于缺乏经过验证的标准和数据匮乏，对这些患者的识别和管理具有挑战性。在本研究中，我们在一组独特的儿科aPL阳性携带者中确定了潜在的其他血栓形成风险因素。
对2001年至2019年在我院就诊的491例儿科CTD患者进行回顾性病历审查。排除在至少12周间隔内没有持续中度至高度滴度aPL的患者。进行单因素分析以评估不同风险因素与血栓形成事件之间的相关性。
该队列纳入了71例患有潜在CTD的aPL阳性儿童。大多数（87%）为女性，西班牙裔（56%）。该队列在诊断结缔组织病时的平均年龄为12.7（标准差2.6）岁，首次aPL阳性的平均年龄为13.3（标准差2.5）岁。平均随访时间为4.3（标准差2.5）年。4例（5.6%）患者发生动脉血栓形成，11例（15.5%）发生静脉血栓形成。57例（80.3%）患者未发生任何血栓栓塞事件。在传统风险因素和内皮损伤迹象中，只有雷诺现象与动脉血栓形成有显著关联（比值比=8.4，95%置信区间1.13 - 111，P = 0.039），高血压或使用抗高血压药物与静脉血栓形成有显著关联（比值比=8.387，95%置信区间1.2 - 94，P = 0.02）。
我们队列的数据表明，雷诺现象是aPL阳性儿科携带者动脉血栓形成的潜在预测因素，而高血压或使用抗高血压药物是静脉血栓形成的潜在预测因素。需要进一步研究儿科aPL谱和血栓形成发展的风险因素，以帮助指导临床医生护理这些具有挑战性的患者。
