动脉炎性前部缺血性视神经病变导致的突发性视力丧失:嗜酸性粒细胞肉芽肿伴多血管炎的罕见表现

A. Faraone, Alberto Fortini, Vanni Borgioli, C. Cappugi, Aldo Lo Forte, Valeria Maria Bottaro, Augusto Vaglio
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摘要

背景:嗜酸性粒细胞肉芽肿伴多血管炎(EGPA)是一种罕见的多系统炎症性疾病,其特征是哮喘、嗜酸性粒细胞增多、肉芽肿或血管炎累及多个器官。虽然眼部受累在 EGPA 患者中并不常见,但也有报道称眼部出现多种表现,如不及时治疗可导致严重的视力损害。病例报告:我们报告了一例 79 岁女性患者的病例,她有哮喘和鼻息肉病史,曾出现低热、轻度精神状态改变和乏力。胸部 X 光片显示双侧间质浸润。实验室检查显示 C 反应蛋白水平升高,嗜酸性粒细胞增多(嗜酸性粒细胞计数为 4.6 x109 cells/l);血液培养和粪便寄生虫检查均呈阴性。就诊四天后,患者报告左眼视力突然严重模糊。眼科检查发现双侧视盘肿胀,视野缺损,左眼更为严重。诊断结果为 EGPA 并发动脉炎性前部缺血性视神经病变(A-AION),同时根据临床表现排除了巨细胞动脉炎的替代或并发诊断。医生立即开始使用大剂量糖皮质激素进行免疫抑制治疗。患者的视力缺陷没有得到改善;然而,两个月后,眼科复查结果显示患者的视力没有恶化。结论:A-AION是EGPA的一种少见但严重的表现形式,需要及时诊断并给予紧急糖皮质激素治疗,以防止视力进一步下降。疾病意识和多学科方法对于加快诊断工作和有效处理与EGPA相关的眼部并发症至关重要。
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Sudden visual loss due to arteritic anterior ischaemic optic neuropathy: A rare manifestation of eosinophilic granulomatosis with polyangiitis
Background: eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of various organs. While the eye is uncommonly affected in patients with EGPA, multiple ophthalmic manifestations have been reported, which can result in serious visual impairment without timely treatment. Case report: we report the case of a 79-year-old woman with a history of asthma and nasal polyps who presented with low-grade fever, mild alteration of mental status, and fatigue. Chest X-ray revealed bilateral interstitial infiltrates. Lab tests showed elevated C-reactive protein level and eosinophilia (eosinophil count, 4.6 x109 cells/l); blood cultures and parasitological examination of stools tested negative. Four days after presentation, the patient reported sudden and severe blurring of vision in her left eye. Ophthalmological examination revealed bilateral swollen optic disc and visual field loss, more severe in the left eye. A diagnosis of EGPA complicated by arteritic anterior ischaemic optic neuropathy (A-AION) was proposed, while an alternative or concurrent diagnosis of giant cell arteritis was ruled out based on clinical picture. Immunosuppressive treatment with high-dose intravenous glucocorticoids was promptly started. The patient’s visual defect did not improve; however, two months later, no worsening was registered on ophthalmic reassessment. Conclusions: A-AION is an infrequent but severe manifestation of EGPA, requiring prompt diagnosis and emergency-level glucocorticoid therapy to prevent any further vision loss. Disease awareness and a multidisciplinary approach are crucial to expedite diagnostic work-up and effective management of EGPA-related ocular complications.
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