Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child
Introduction: A chronic active Epstein–Barr virus (EBV) infection (CAEBV), which is characterized by persistent “infectious mononucleosis-like” symptoms, can lead to cardiovascular complications, including coronary artery aneurysms. No published studies have reported an occurrence of chronic EB viru...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2019-06-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fped.2019.00219/full |
| _version_ | 1811241413051940864 |
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| author | Hongjun Ba Lingling Xu Huimin Peng Yuese Lin Xuandi Li Huishen Wang Youzhen Qin |
| author_facet | Hongjun Ba Lingling Xu Huimin Peng Yuese Lin Xuandi Li Huishen Wang Youzhen Qin |
| author_sort | Hongjun Ba |
| collection | DOAJ |
| description | Introduction: A chronic active Epstein–Barr virus (EBV) infection (CAEBV), which is characterized by persistent “infectious mononucleosis-like” symptoms, can lead to cardiovascular complications, including coronary artery aneurysms. No published studies have reported an occurrence of chronic EB virus infection in conjunction with systemic vasculitis and pulmonary hypertension.Case Presentation: Herein, we present a case of a 9-year-old boy with CAEBV, associated with pulmonary arterial hypertension (PAH) and systemic vasculitis. Recurrent skin ulcers were a major early clinical manifestation in this case. The histopathological examination of a dermal biopsy sample from the lesions revealed vasculitis, and the in-situ hybridization test was positive for EBV-encoded small RNA.Results: The patient was administered immunosuppressants (prednisolone and cyclophosphamide) and targeted drugs (sildenafil and bosentan) to control the pulmonary pressure. This combination therapy decreased the systolic pulmonary arterial pressure to 40 mm Hg (on echocardiography), and the N-terminal pro b-type natriuretic peptide level also reduced to 62.3 pg/ml. After discontinuation of prednisone, the child developed shortness of breath, edema, and oliguria. He was again started on prednisone, with an addition of thalidomide. Sildenafil was replaced by riociguat, due to the side effect of penile erection. The patient is being followed up every 2 months at the clinic. The most recent follow-up visit was 2 weeks before this report was written, during which, the child was observed to have no rash, shortness of breath, edema, and other symptoms. Written informed consent was obtained from the parents for the publication of this case report.Conclusion: A CAEBV should be considered among the differential diagnoses while managing a pediatric patient with secondary PAH and systemic vasculitis. However, elucidation of its potential pathophysiological mechanisms requires further study. |
| first_indexed | 2024-04-12T13:35:46Z |
| format | Article |
| id | doaj.art-ab8647662cb247559e3aa4855bdd9042 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-12T13:35:46Z |
| publishDate | 2019-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-ab8647662cb247559e3aa4855bdd90422022-12-22T03:31:01ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602019-06-01710.3389/fped.2019.00219453327Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a ChildHongjun Ba0Lingling Xu1Huimin Peng2Yuese Lin3Xuandi Li4Huishen Wang5Youzhen Qin6Department of Pediatric Cardiology, Heart Center, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, ChinaDepartment of Pediatrics, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, ChinaDepartment of Pediatric Cardiology, Heart Center, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, ChinaDepartment of Pediatric Cardiology, Heart Center, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, ChinaDepartment of Pediatric Cardiology, Heart Center, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, ChinaDepartment of Pediatric Cardiology, Heart Center, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, ChinaDepartment of Pediatric Cardiology, Heart Center, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, ChinaIntroduction: A chronic active Epstein–Barr virus (EBV) infection (CAEBV), which is characterized by persistent “infectious mononucleosis-like” symptoms, can lead to cardiovascular complications, including coronary artery aneurysms. No published studies have reported an occurrence of chronic EB virus infection in conjunction with systemic vasculitis and pulmonary hypertension.Case Presentation: Herein, we present a case of a 9-year-old boy with CAEBV, associated with pulmonary arterial hypertension (PAH) and systemic vasculitis. Recurrent skin ulcers were a major early clinical manifestation in this case. The histopathological examination of a dermal biopsy sample from the lesions revealed vasculitis, and the in-situ hybridization test was positive for EBV-encoded small RNA.Results: The patient was administered immunosuppressants (prednisolone and cyclophosphamide) and targeted drugs (sildenafil and bosentan) to control the pulmonary pressure. This combination therapy decreased the systolic pulmonary arterial pressure to 40 mm Hg (on echocardiography), and the N-terminal pro b-type natriuretic peptide level also reduced to 62.3 pg/ml. After discontinuation of prednisone, the child developed shortness of breath, edema, and oliguria. He was again started on prednisone, with an addition of thalidomide. Sildenafil was replaced by riociguat, due to the side effect of penile erection. The patient is being followed up every 2 months at the clinic. The most recent follow-up visit was 2 weeks before this report was written, during which, the child was observed to have no rash, shortness of breath, edema, and other symptoms. Written informed consent was obtained from the parents for the publication of this case report.Conclusion: A CAEBV should be considered among the differential diagnoses while managing a pediatric patient with secondary PAH and systemic vasculitis. However, elucidation of its potential pathophysiological mechanisms requires further study.https://www.frontiersin.org/article/10.3389/fped.2019.00219/fullchronic active Epstein–Barr virus infectioncoronary artery aneurysmpulmonary arterial hypertensionvasculitiscardiac insufficiency |
| spellingShingle | Hongjun Ba Lingling Xu Huimin Peng Yuese Lin Xuandi Li Huishen Wang Youzhen Qin Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child Frontiers in Pediatrics chronic active Epstein–Barr virus infection coronary artery aneurysm pulmonary arterial hypertension vasculitis cardiac insufficiency |
| title | Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child |
| title_full | Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child |
| title_fullStr | Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child |
| title_full_unstemmed | Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child |
| title_short | Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child |
| title_sort | chronic active epstein barr virus infection with systemic vasculitis and pulmonary arterial hypertension in a child |
| topic | chronic active Epstein–Barr virus infection coronary artery aneurysm pulmonary arterial hypertension vasculitis cardiac insufficiency |
| url | https://www.frontiersin.org/article/10.3389/fped.2019.00219/full |
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