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嗜酸性肉芽肿性多血管炎:一例报告

Eosinophilic Granulomatosis With Polyangiitis: A Case Report.

作者信息

Robinson Ciji, Minhas Jasdeep S, Kisule Abraham, Zebda Hazem

机构信息

Internal Medicine, Henry Ford Health System, Jackson, USA.

Medicine, St. George's University School of Medicine, St. George's, GRD.

出版信息

Cureus. 2024 Apr 13;16(4):e58211. doi: 10.7759/cureus.58211. eCollection 2024 Apr.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of necrotizing small-to-medium vessel vasculitis that can be associated with antineutrophil cytoplasmic antibody (ANCA) positivity, asthma, and eosinophilia. We present the case of a 65-year-old male with a past medical history of asthma who presented to the emergency department with bilateral upper and lower extremity paresthesias, as well as right foot drop, persisting for a two-week duration. His lab work revealed leukocytosis of 20.6 K/uL with 12.36 K/uL of absolute eosinophils as well as elevated inflammatory markers with an erythrocyte sedimentation rate of 32 mm/hr and CRP of 7.3 mg/dL. Both c-ANCA and p-ANCA titers were also elevated at 1:320. An eventual MRI of the entire spine did not reveal any neurologic or anatomic lesions to explain the patient's symptoms. CT imaging was also remarkable for airspace opacities involving the anterior right and bilateral lower posterior lung regions, as well as pansinusitis. A nerve biopsy showed axonopathy as well as evidence of healed vasculitis. Pulse dose steroids were started, which conferred benefits to the patient after other forms of treatment were unsuccessful. Given the rarity of EGPA, we think it is important to add new cases to the literature with a thorough discussion of the steps leading up to how the diagnosis was made.

摘要

嗜酸性肉芽肿性多血管炎(EGPA)是一种罕见的坏死性中小血管血管炎,可伴有抗中性粒细胞胞浆抗体(ANCA)阳性、哮喘和嗜酸性粒细胞增多。我们报告一例65岁男性患者,有哮喘病史,因双侧上下肢感觉异常以及右足下垂就诊于急诊科,症状持续两周。他的实验室检查显示白细胞计数为20.6K/uL,绝对嗜酸性粒细胞计数为12.36K/uL,炎症指标升高,红细胞沉降率为32mm/hr,C反应蛋白为7.3mg/dL。c-ANCA和p-ANCA滴度也升高至1:320。最终对整个脊柱进行的MRI检查未发现任何神经或解剖学病变来解释患者的症状。CT成像还显示右前肺和双侧下后肺区域有气腔混浊,以及全鼻窦炎。神经活检显示轴索性神经病以及愈合性血管炎的证据。在其他治疗方法均未成功后,开始使用大剂量脉冲类固醇治疗,该治疗使患者受益。鉴于EGPA的罕见性,我们认为向文献中添加新病例并对诊断过程进行全面讨论非常重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/11089492/920025e6f7b5/cureus-0016-00000058211-i01.jpg

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