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“一例表现为四肢瘫痪的嗜酸性肉芽肿性多血管炎”:临床与诊断报告

"A Case of Eosinophilic Granulomatosis With Polyangiitis Presenting as Quadriplegia": A Clinical and Diagnostic Report.

作者信息

Inayat Waqas, Iqbal Sehba, Adnan Fatma, Ali Amjad, Zeb Alam

机构信息

Khyber Medical College, Khyber Teaching Hospital, Peshawar, Pakistan.

Department of Rheumatology, Khyber Teaching Hospital, Peshawar, Pakistan.

出版信息

J Investig Med High Impact Case Rep. 2025 Jan-Dec;13:23247096251374513. doi: 10.1177/23247096251374513. Epub 2025 Sep 18.

DOI:10.1177/23247096251374513
PMID:40968472
Abstract

An antineutrophil cytoplasmic antibody-associated small artery vasculitis that primarily affects the skin, lungs, and heart is called eosinophilic granulomatosis with polyangiitis (EGPA). It typically begins with initial respiratory symptoms like asthma and chronic sinusitis, and later progresses to systemic vasculitic issues that are linked to increased levels of eosinophils in the blood and tissues. While both central nervous system (CNS) and peripheral nervous systems can be affected, CNS involvement is rare. Nerve damage from vasculitis in EGPA usually appears as mononeuritis multiplex and is infrequently seen as polyradiculopathy. Here, we discuss an unusual case where a patient with EGPA presented with worsening asthma, a skin rash, and a sudden quadriplegia due to involvement of the cervical spine. After all relevant investigations and findings, the patient was treated with IV rituximab along with IV corticosteroids and oral azathioprine which improved the condition including the paralysis.

摘要

一种主要影响皮肤、肺部和心脏的抗中性粒细胞胞浆抗体相关的小动脉血管炎被称为嗜酸性肉芽肿性多血管炎(EGPA)。它通常始于初始的呼吸道症状,如哮喘和慢性鼻窦炎,随后进展为与血液和组织中嗜酸性粒细胞水平升高相关的全身性血管炎问题。虽然中枢神经系统(CNS)和周围神经系统均可受累,但CNS受累较为罕见。EGPA中血管炎导致的神经损伤通常表现为多发性单神经炎,很少表现为多发性神经根病。在此,我们讨论一例不寻常的病例,一名EGPA患者因颈椎受累出现哮喘加重、皮疹和突然四肢瘫痪。经过所有相关检查和结果分析后,该患者接受了静脉注射利妥昔单抗以及静脉注射皮质类固醇和口服硫唑嘌呤治疗,病情包括瘫痪均得到改善。

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