Background/Purpose: While pediatric systemic lupus erythematosus (pSLE) represents only 20% of all SLE cases, pSLE patients often have more aggressive disease with multi-organ involvement. These patients can be at significant risk for thrombotic events due to systemic inflammation, vasculopathy, antiphospholipid antibodies (aPLs) and nephrotic features. Data detailing the timing and nature of thrombotic events in pSLE patients are limited. The objective of this study was to further characterize thrombotic events in a pSLE patient cohort at a large academic medical center.

Methods: A retrospective chart review was completed for patients with pSLE who had a documented thrombotic event. All patients fulfilled the American College of Rheumatology (ACR) classification criteria for SLE. Descriptive statistics were utilized for this preliminary analysis.

Results: Of the 402 patients in this pSLE cohort, there were a total of 45 thrombotic events in 28 patients. The cohort was 89% female, 50% African American, Non-Hispanic and 43% Caucasian, Hispanic. Mean age at pSLE diagnosis was 12.9 years, range was from 6.7 to 17.8 years. 71% (20) had positive APLs at pSLE diagnosis: 60% lupus anticoagulant (LA), 70% anticardiolipin (aCL), and 45% antiβ2glycoprotein1 (aβ2GP1). Mean age at first thrombotic event was 14.9 years, range was from 7.8 to 22.9 years. There was a mean difference of 2.0 years from pSLE diagnosis to thrombotic event, with a range of -4.4 to 10.3 years. Fifteen patients (54%) were diagnosed with pSLE at the time of the initial thrombotic event. Of the 45 thrombotic events, 33% (15) were arterial and 66% (30) were venous. Arterial events included 10 cerebrovascular accidents and 4 transient ischemic attacks. Venous events included 16 deep vein thromboses (DVT), 2 pulmonary emboli (PE) without radiographically identified DVT, and 3 combined DVT/PEs. Seven patients (25%) had multiple thrombotic events. At the time of the thrombotic event, 56% were aPL positive (60% LA, 72% aCL, and 40% aβ2GP1), 11% were negative, and 33% were either not tested or the data was not available.

Conclusion: The majority of the thrombotic events in this cohort were venous, and a single occurrence. More than half of the patients were diagnosed with pSLE as a result of the presenting thrombotic event. Most patients had positive antiphospholipid antibodies both at pSLE diagnosis and at the time of the thrombotic event. Further research plans for this dataset include further analysis of predictive factors for thrombosis and characterizing the variation in establishing initial thrombotic risk, surveillance and management.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/thrombotic-events-in-pediatric-systemic-lupus-erythematosus-a-preliminary-analysis-of-a-large-single-center-cohort/