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白塞综合征患者并发心内膜炎、心内血栓及肺动脉瘤

Endocarditis, Intra-cardiac Thrombus, and Pulmonary Artery Aneurysm in a Patient with Behcet's Syndrome.

作者信息

Yildiz Fatih, Kelle Bayram, Erken Eren

机构信息

Division of Rheumatology, Department of Internal Medicine, Kahramanmaras Sutcu Imam University Faculty of Medicine, Kahramanmaras, Türkiye.

Department of Physical Medicine and Rehabilitation, Cukurova University Faculty of Medicine, Adana, Türkiye.

出版信息

Sisli Etfal Hastan Tip Bul. 2024 Jun 28;58(2):244-248. doi: 10.14744/SEMB.2023.80000. eCollection 2024.

DOI:10.14744/SEMB.2023.80000
PMID:39021690
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11249990/
Abstract

Behçet's Syndrome (BS) is a chronic vasculitis of unknown etiology. Arterial involvement occurring in the pulmonary artery is associated with poor prognosis. It may cause pulmonary thrombus (PTE) and aneurysm (PAA) which may also lead to a rare complication, intracardiac thrombus. PAA and PTE can be complications of BS and are associated with high morbidity and mortality. A 30-year-old male patient had a fever of 38.4°C, recurrent oral-genital ulcers, shortness of breath, cough, and sputum. In this case report, medical history, clinical and laboratory examinations, radiography, echocardiography, and computer tomography imaging examinations were performed. PAA, PTE, intracardiac and left popliteal vein thrombosis, and infective endocarditis were present. The patient was diagnosed with BS according to the International Study Group criteria. Surgery was performed for intracardiac thrombus. Vegetation within the thrombus was demonstrated histopathologically. The patient's clinical condition and laboratory tests improved with intervention and medical treatments. The patient with BS, PAA, PTE, intracardiac thrombus, and infective endocarditis was successfully treated with pulmonary embolization, antibiotics, and systemic immunosuppression, despite its rarity, poor prognosis, and high morbidity and mortality rates.

摘要

白塞病(BS)是一种病因不明的慢性血管炎。肺动脉受累与预后不良相关。它可能导致肺血栓(PTE)和动脉瘤(PAA),这也可能导致一种罕见的并发症——心内血栓。PAA和PTE可能是白塞病的并发症,且与高发病率和死亡率相关。一名30岁男性患者出现38.4°C发热、复发性口腔生殖器溃疡、呼吸急促、咳嗽和咳痰。在本病例报告中,进行了病史、临床和实验室检查、放射学检查、超声心动图检查以及计算机断层扫描成像检查。发现存在PAA、PTE、心内和左腘静脉血栓形成以及感染性心内膜炎。根据国际研究组标准,该患者被诊断为白塞病。对心内血栓进行了手术治疗。组织病理学检查显示血栓内有赘生物。经过干预和药物治疗,患者的临床状况和实验室检查结果有所改善。尽管患有白塞病、PAA、PTE、心内血栓和感染性心内膜炎的情况罕见、预后不良且发病率和死亡率高,但通过肺栓塞、抗生素和全身免疫抑制治疗,该患者成功治愈。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd2e/11249990/393770afe364/SEMB-58-244-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd2e/11249990/d1b14b02cacb/SEMB-58-244-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd2e/11249990/393770afe364/SEMB-58-244-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd2e/11249990/d1b14b02cacb/SEMB-58-244-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd2e/11249990/393770afe364/SEMB-58-244-g002.jpg

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本文引用的文献

1
Pulmonary artery thrombosis as the first presentation of Behçet's syndrome: a case report and review of the literature.肺动脉血栓形成作为白塞综合征的首发表现:病例报告及文献复习。
J Med Case Rep. 2021 Jun 22;15(1):322. doi: 10.1186/s13256-021-02931-1.
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Cardiac and vascular complications of Behçet disease in the Tunisian context: clinical characteristics and predictive factors.贝切特病在突尼斯语境下的心脏和血管并发症:临床特征和预测因素。
Adv Rheumatol. 2018 Oct 1;58(1):32. doi: 10.1186/s42358-018-0032-x.
3
Vascular Behçet's syndrome: an update.
血管型贝赫切特综合征:最新进展。
Intern Emerg Med. 2019 Aug;14(5):645-652. doi: 10.1007/s11739-018-1991-y. Epub 2018 Nov 29.
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Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behcet's disease: A multicenter observational study of 18 patients.抗 TNF-α 治疗 Behcet 病重度、难治性大血管受累的疗效:一项多中心观察性研究的 18 例患者。
Clin Immunol. 2018 Dec;197:54-59. doi: 10.1016/j.clim.2018.08.004. Epub 2018 Aug 18.
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2018 update of the EULAR recommendations for the management of Behçet's syndrome.2018 年更新的欧洲抗风湿病联盟白塞病治疗推荐。
Ann Rheum Dis. 2018 Jun;77(6):808-818. doi: 10.1136/annrheumdis-2018-213225. Epub 2018 Apr 6.
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Behçet disease with vascular involvement: effects of different therapeutic regimens on the incidence of new relapses.伴有血管受累的白塞病:不同治疗方案对新复发率的影响。
Medicine (Baltimore). 2015 Feb;94(6):e494. doi: 10.1097/MD.0000000000000494.
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A rare but serious manifestation of Behçet's disease: intracardiac thrombus in 22 patients.白塞病一种罕见但严重的表现:22例患者出现心内血栓形成。
Clin Exp Rheumatol. 2014 Jul-Aug;32(4 Suppl 84):S87-92. Epub 2014 Jul 28.
8
Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients.白塞病中的肺动脉受累及相关肺部疾病:47例患者系列研究
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Behçet's Syndrome and Thrombosis.白塞综合征与血栓形成。
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Intracardiac thrombus and pulmonary aneurysms in an adolescent with Behçet disease.一名患有白塞病的青少年的心脏内血栓和肺动脉瘤
Rheumatol Int. 2009 Mar;29(5):575-7. doi: 10.1007/s00296-008-0730-5. Epub 2008 Oct 11.