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产后发病的高安动脉炎伴主动脉夹层形成。

Postpartum onset Takayasu's arteritis presenting with aortic dissection.

作者信息

Yamamoto Hirotaka, Taniguchi Yoshinori, Miura Yujiro, Kobayashi Shigeto

机构信息

Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi Medical School Hospital, Kochi University, 185-1 Kohasu, Oko-cho, Nankoku, Kochi 783-8505, Japan.

Department of Cardiovascular Surgery, Kochi Medical School Hospital, Kochi University, 185-1 Kohasu, Oko-cho, Nankoku, Kochi 783-8505, Japan.

出版信息

Oxf Med Case Reports. 2024 Jul 30;2024(7):omae078. doi: 10.1093/omcr/omae078. eCollection 2024 Jul.

Abstract

Takayasu's arteritis (TA), also known as pulseless disease and young female arteritis, is a chronic inflammatory large-vessel vasculitis (LVV). TA is pathologically characterized by arterial wall thickening, stenotic/occlusive lesions, aneurysm formation, and dissection. TA usually develops between 20 and 30 years of age. However, pregnancy and puerperium can affect the immune system, and several cases of postpartum onset or flare-up of TA have been reported. Herein, we report an extremely rare case of postpartum-onset TA complicated by aortic dissection. This is a case of Postpartum onset Takayasu's arteritis presenting with aortic dissection. A 34-year-old healthy woman was performed cesarean section. After 2 weeks, she presented with chest pain and fever, followed by mild dysphagia and hoarseness. Laboratory findings showed C-reactive protein (CRP) 21.61 mg/dl and computed tomography (CT) demonstrated thickening of the vessel wall of mainly ascending aorta. 18F-fluorodeoxyglucose (FDG)-position emission tomography (PET)/CT revealed high FDG uptake in the same areas. We diagnosed with TA and steroid pulse therapy was started. However, five days after treatment, the patient developed worsening symptoms of hoarseness. A contrast-enhanced CT showed Stanford A type dissection, and emergency artificial vessel replacement was performed. The specimen from surgical resection of the ascending aorta suggested active TA associated with dissection. The prednisolone dosage was gradually tapered with tocilizumab. Then, her symptoms and laboratory findings improved. It is important to recall the onset of TA and/or arterial dissection, when patients develop chest pain and hoarseness in the postpartum period.

摘要

高安动脉炎(TA),又称无脉症和青年女性动脉炎,是一种慢性炎症性大血管血管炎(LVV)。TA的病理特征为动脉壁增厚、狭窄/闭塞性病变、动脉瘤形成和夹层。TA通常在20至30岁之间发病。然而,妊娠和产褥期会影响免疫系统,已有数例TA产后发病或病情加重的报道。在此,我们报告一例极为罕见的产后发病TA合并主动脉夹层病例。这是一例产后发病的高安动脉炎伴主动脉夹层病例。一名34岁健康女性接受了剖宫产手术。术后2周,她出现胸痛和发热,随后出现轻度吞咽困难和声音嘶哑。实验室检查结果显示C反应蛋白(CRP)为21.61mg/dl,计算机断层扫描(CT)显示主要是升主动脉的血管壁增厚。18F-氟脱氧葡萄糖(FDG)-正电子发射断层扫描(PET)/CT显示相同区域FDG摄取增高。我们诊断为TA并开始使用类固醇脉冲疗法。然而,治疗五天后,患者声音嘶哑症状加重。增强CT显示斯坦福A型夹层,遂进行了急诊人工血管置换术。升主动脉手术切除标本提示为与夹层相关的活动性TA。泼尼松龙剂量逐渐减量,并加用托珠单抗。随后,她的症状和实验室检查结果有所改善。产后患者出现胸痛和声音嘶哑时,回顾TA和/或动脉夹层的发病情况很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd05/11289832/5f10e348e948/omae078f1.jpg

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