Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management
Abstract 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...
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| Format: | Article |
| Language: | English |
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BMC
2025-01-01
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| Series: | Pediatric Rheumatology Online Journal |
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| Online Access: | https://doi.org/10.1186/s12969-024-01054-3 |
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| author | Batuhan Küçükali Merve Yazol Çisem Yıldız Büşra Acun Nuran Belder Nihal Karaçayır Merve Kutlar Pelin Esmeray Şenol Zühre Kaya Deniz Gezgin Yıldırım Sevcan A. Bakkaloğlu |
| author_facet | Batuhan Küçükali Merve Yazol Çisem Yıldız Büşra Acun Nuran Belder Nihal Karaçayır Merve Kutlar Pelin Esmeray Şenol Zühre Kaya Deniz Gezgin Yıldırım Sevcan A. Bakkaloğlu |
| author_sort | Batuhan Küçükali |
| collection | DOAJ |
| description | Abstract 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. |
| format | Article |
| id | doaj-art-85e02213557e4fc0abcc7c2be41d5679 |
| institution | Kabale University |
| issn | 1546-0096 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj-art-85e02213557e4fc0abcc7c2be41d56792025-01-05T12:11:16ZengBMCPediatric Rheumatology Online Journal1546-00962025-01-0123111010.1186/s12969-024-01054-3Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing managementBatuhan Küçükali0Merve Yazol1Çisem Yıldız2Büşra Acun3Nuran Belder4Nihal Karaçayır5Merve Kutlar6Pelin Esmeray Şenol7Zühre Kaya8Deniz Gezgin Yıldırım9Sevcan A. Bakkaloğlu10Department of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Radiology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Hematology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityDepartment of Pediatric Rheumatology, Faculty of Medicine, Gazi UniversityAbstract 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.https://doi.org/10.1186/s12969-024-01054-3EGPAThromboembolismPulmonary embolismChurg-Strauss SyndromeEosinophilic Granulomatosis with Polyangiitis |
| spellingShingle | Batuhan Küçükali Merve Yazol Çisem Yıldız Büşra Acun Nuran Belder Nihal Karaçayır Merve Kutlar Pelin Esmeray Şenol Zühre Kaya Deniz Gezgin Yıldırım Sevcan A. Bakkaloğlu Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management Pediatric Rheumatology Online Journal EGPA Thromboembolism Pulmonary embolism Churg-Strauss Syndrome Eosinophilic Granulomatosis with Polyangiitis |
| title | Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management |
| title_full | Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management |
| title_fullStr | Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management |
| title_full_unstemmed | Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management |
| title_short | Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management |
| title_sort | massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis a case based review emphasizing management |
| topic | EGPA Thromboembolism Pulmonary embolism Churg-Strauss Syndrome Eosinophilic Granulomatosis with Polyangiitis |
| url | https://doi.org/10.1186/s12969-024-01054-3 |
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