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Multiple hepatic aneurysms and dry gangrene of fingertips in eosinophilic granulomatosis with polyangiitis: a case report
Allergy, Asthma & Clinical Immunology ( IF 2.6 ) Pub Date : 2020-10-15 , DOI: 10.1186/s13223-020-00484-4
Eunsil Koh 1 , Noeul Kang 1 , Jin-Young Lee 2 , Duk-Kyung Kim 1 , Young Soo Do 3 , Byung-Jae Lee 1 , Dong-Chull Choi 1
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Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis mainly affecting small-sized arteries. Involvement of medium-sized vessels is very rare in EGPA. Here we present the case of a patient with EGPA who showed multiple hepatic aneurysms and distal gangrene. A known EGPA patient visited to the emergency room (ER) with abrupt squeezing abdominal pain. She had suffered from gangrene in the fingertips of both hands for 1 year because of arterial thrombosis associated with hypereosinophilia. However, her absolute eosinophil count in the ER was 1120 cells/µL. An abdomen-pelvis CT demonstrated subcapsular hematoma in the right hepatic lobe. A celiac angiogram demonstrated multiple sized aneurysms in both hepatic lobes and some aneurysms in S7 and S8 were huge, more than 1 cm in size. The shape of the small aneurysms resembled a string of beads, as in polyarteritis nodosa. Given the clinical situation, emergency embolization was performed. Before this patient visited to the ER, she had been treated with a high dose of systemic corticosteroid, azathioprine, and cyclophosphamide. After addition of mepolizumab, the eosinophil count remained stable state with a near zero percentage of total white blood cell count. Aneurysm and gangrene resulting from the involvement of medium-sized vessels can occur in EGPA. Destruction of vessels might occur even if eosinophil count is below 1500 cells/µL. If involvement of medium-sized arteries is suspected, thorough investigation to identify the involved organs and prompt management are needed to prevent fatal complications.

中文翻译:

嗜酸性肉芽肿伴多发性血管炎多发性肝动脉瘤及指尖干性坏疽1例报告

嗜酸性肉芽肿性多血管炎(EGPA)是一种主要累及小动脉的系统性坏死性血管炎。EGPA 中很少见中型血管受累。在这里,我们介绍了一个 EGPA 患者的病例,他表现出多个肝动脉瘤和远端坏疽。一位已知的 EGPA 患者因突然挤压性腹痛就诊于急诊室 (ER)。由于嗜酸性粒细胞增多相关的动脉血栓形成,她曾患过双手指尖坏疽 1 年。然而,她在 ER 中的绝对嗜酸性粒细胞计数为 1120 个细胞/µL。腹部-盆腔 CT 显示右肝叶包膜下血肿。腹腔血管造影显示两个肝叶都有多个大小的动脉瘤,S7 和 S8 的一些动脉瘤很大,超过 1 厘米。小动脉瘤的形状类似于一串珠子,如结节性多动脉炎。鉴于临床情况,进行了紧急栓塞。在这名患者就诊于急诊室之前,她接受了大剂量的全身性皮质类固醇、硫唑嘌呤和环磷酰胺治疗。添加美泊利单抗后,嗜酸性粒细胞计数保持稳定状态,占总白细胞计数的百分比接近零。中型血管受累导致的动脉瘤和坏疽可发生在 EGPA 中。即使嗜酸性粒细胞计数低于 1500 个细胞/µL,也可能发生血管破坏。如果怀疑有中型动脉受累,则需要彻底调查以确定受累器官并及时处理,以防止致命的并发症。鉴于临床情况,进行了紧急栓塞。在这名患者就诊于急诊室之前,她接受了大剂量的全身性皮质类固醇、硫唑嘌呤和环磷酰胺治疗。添加美泊利单抗后,嗜酸性粒细胞计数保持稳定状态,占总白细胞计数的百分比接近零。中型血管受累导致的动脉瘤和坏疽可发生在 EGPA 中。即使嗜酸性粒细胞计数低于 1500 个细胞/µL,也可能发生血管破坏。如果怀疑有中型动脉受累,则需要彻底调查以确定受累器官并及时处理,以防止致命的并发症。鉴于临床情况,进行了紧急栓塞。在这名患者就诊于急诊室之前,她接受了大剂量的全身性皮质类固醇、硫唑嘌呤和环磷酰胺治疗。添加美泊利单抗后,嗜酸性粒细胞计数保持稳定状态,占总白细胞计数的百分比接近零。中型血管受累导致的动脉瘤和坏疽可发生在 EGPA 中。即使嗜酸性粒细胞计数低于 1500 个细胞/µL,也可能发生血管破坏。如果怀疑有中型动脉受累,则需要彻底调查以确定受累器官并及时处理,以防止致命的并发症。硫唑嘌呤和环磷酰胺。添加美泊利单抗后,嗜酸性粒细胞计数保持稳定状态,占总白细胞计数的百分比接近零。中型血管受累导致的动脉瘤和坏疽可发生在 EGPA 中。即使嗜酸性粒细胞计数低于 1500 个细胞/µL,也可能发生血管破坏。如果怀疑有中型动脉受累,则需要彻底调查以确定受累器官并及时处理,以防止致命的并发症。硫唑嘌呤和环磷酰胺。添加美泊利单抗后,嗜酸性粒细胞计数保持稳定状态,占总白细胞计数的百分比接近零。中型血管受累导致的动脉瘤和坏疽可发生在 EGPA 中。即使嗜酸性粒细胞计数低于 1500 个细胞/µL,也可能发生血管破坏。如果怀疑有中型动脉受累,则需要彻底调查以确定受累器官并及时处理,以防止致命的并发症。即使嗜酸性粒细胞计数低于 1500 个细胞/µL,也可能发生血管破坏。如果怀疑有中型动脉受累,则需要彻底调查以确定受累器官并及时处理,以防止致命的并发症。即使嗜酸性粒细胞计数低于 1500 个细胞/µL,也可能发生血管破坏。如果怀疑有中型动脉受累,则需要彻底调查以确定受累器官并及时处理,以防止致命的并发症。
更新日期:2020-10-15
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