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Identifying additional risk factors for arterial and venous thrombosis among pediatric antiphospholipid antibodies

Elizabeth Sloan^1,2,3, Tracey Wright^1,2,3, Yu Zuo⁴

¹Division of Pediatric Rheumatology, Department of Pediatrics, UT Southwestern Medical Center, Dallas, USA.

|March 19, 2021

Insights

In pediatric patients with antiphospholipid antibodies (aPL) and connective tissue disease (CTD), Raynaud's phenomenon may predict arterial clots, while hypertension predicts venous clots. Further research is needed for these aPL carriers.

Area of Science:

Pediatric Rheumatology
Hematology
Immunology

Background:

Antiphospholipid antibodies (aPL) are reported in children, but thrombotic risks in pediatric patients with aPL positivity are understudied.
Identifying and managing pediatric patients with aPL and connective tissue diseases (CTD) is challenging due to limited data and validated criteria.
This study identifies potential additional risk factors for thrombosis in pediatric aPL-positive carriers with CTD.

Purpose of the Study:

To investigate risk factors for thrombosis in pediatric patients with antiphospholipid antibodies (aPL) and connective tissue disease (CTD).
To identify potential predictors of arterial and venous thrombosis in this specific pediatric cohort.

Main Methods:

Retrospective chart review of 491 pediatric patients with CTD from 2001-2019.
Inclusion criteria: persistently moderate to high titer aPL at least 12 weeks apart.
Univariate analysis to correlate risk factors with thrombotic events.

Main Results:

Seventy-one aPL-positive pediatric CTD patients were included (87% female, 56% Hispanic).
Arterial thrombosis occurred in 5.6% and venous thrombosis in 15.5% of patients.
Raynaud's phenomenon was associated with arterial thrombosis (OR=8.4), and hypertension/antihypertensive use with venous thrombosis (OR=8.387).

Conclusions:

Raynaud's phenomenon may predict arterial thrombosis in pediatric aPL carriers with CTD.
Hypertension or antihypertensive use may predict venous thrombosis in this population.
Further research on pediatric aPL profiles and thrombosis risk factors is crucial for clinical guidance.

Read full analysis

Abstract

Background:

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.

Methods:

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.

Results:

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).

Conclusion:

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.

Keywords:

Thrombosis antiphospholipid syndrome systemic lupus erythematosus

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