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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a
Batuhan Küçükali1, Merve Yazol2, Çisem Yıldız3
1Department of Pediatric Rheumatology, Faculty of Medicine, Gazi University, Ankara, Besevler, 06500, Turkey. Batuhan.kucukali@gazi.edu.tr.
Insights
Pediatric Eosinophilic Granulomatosis with Polyangiitis (EGPA) patients face high risks of arterial and venous thromboembolism (AVTE). Treatment often requires anticoagulation and immunosuppression, with potential for good outcomes needing further study.
Area of Science:
- Pediatric Rheumatology
- Hematology
- Immunology
Background:
- Pediatric Eosinophilic Granulomatosis with Polyangiitis (EGPA) patients have an elevated risk of arterial and venous thromboembolism (AVTE).
- The precise mechanisms driving AVTE in pediatric EGPA are not fully understood, but eosinophils are implicated.
- Current clinical guidelines offer limited evidence-based recommendations for anticoagulant and antiplatelet therapies in pediatric EGPA-related AVTE.
Purpose of the Study:
- To raise clinician awareness of AVTE in pediatric EGPA by presenting a case study.
- To review and analyze existing literature on pediatric AVTE in EGPA.
- To evaluate management strategies and provide recommendations for this challenging complication.
Main Methods:
- Case report of a pediatric EGPA patient with deep venous thrombosis and pulmonary thromboembolism.
- Comprehensive literature review of pediatric AVTE cases associated with EGPA.
- Analysis of management strategies and current practice guidelines.
Main Results:
- AVTE events in EGPA predominantly occur during heightened disease activity.
- Thrombocytopenia due to consumption is a notable finding in EGPA patients with VTE, contrary to expectations during exacerbation.
- Venous thrombosis management typically involves a combination of anticoagulation and immunosuppressive therapy.
Conclusions:
- The review suggests a generally favorable prognosis for AVTE in pediatric EGPA, though limited case numbers preclude definitive conclusions.
- Further research is needed to investigate the efficacy of eosinophil-targeted therapies, such as mepolizumab, for AVTE.
- Future studies should also clarify the roles of anticoagulation and antiplatelet treatments in managing AVTE in this population.
Background:
Pediatric patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA) are at an increased risk of arterial and venous thromboembolism (AVTE). Although the exact mechanisms underlying AVTE remain unclear, eosinophils play a pivotal role in AVTE.
Main Body:
Current guidelines lack evidence-based recommendations, particularly concerning anticoagulant and antiplatelet treatments for this condition. Herein, we document a pediatric EGPA patient with deep venous thrombosis presenting with massive pulmonary thromboembolism during a relapse, treated with immunosuppressive and anticoagulant therapy to raise awareness among clinicians. Additionally, we performed a literature review to highlight various aspects of pediatric AVTE. Moreover, we evaluated the management strategies employed for the patients identified in the literature review and summarized the current practice guidelines regarding pediatric EGPA patients with AVTE to provide recommendations to clinicians on the management of this challenging complication.
Conclusions:
Most AVTE events occur during periods of high disease activity. Notably, EGPA patients with VTE often present with thrombocytopenia due to consumption, a finding not typically expected during disease exacerbation. Venous thrombosis generally requires both anticoagulation and immunosuppressive treatment. Although our review indicates a favorable prognosis for AVTE, the small number of reported cases prevents us from drawing definitive conclusions. Future studies should explore the efficacy of mepolizumab and other eosinophil-targeted therapies for AVTE, in addition to investigating the roles of anticoagulation and antiplatelet treatments.
