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坏死性巩膜炎作为嗜酸性肉芽肿性多血管炎的一种表现:一例报告

Necrotizing Scleritis as a Manifestation of Eosinophilic Granulomatosis With Polyangiitis: A Case Report.

作者信息

Arenas-Beltran Mayra Alejandra, Muñoz Velandia Oscar Mauricio

机构信息

Internal Medicine Resident, Pontificia Universidad Javeriana, Bogota, Colombia.

Attending Professor, Internal Medicine Department, Pontificia Universidad Javeriana, Hospital Universitario San Ignacio, Bogota, Colombia.

出版信息

Clin Med Insights Case Rep. 2023 Mar 10;16:11795476231161167. doi: 10.1177/11795476231161167. eCollection 2023.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitis characterized by the development of necrotizing granulomas rich in eosinophils and vasculitis of small and medium vessels, with compromise of the respiratory tract, peripheral nervous system, and least frequent ocular involvement. We report the case of a 54-year-old Caucasian man with a history of EGPA who presented ocular pain, red eye, vision loss, and evidence of scleral slimming compatible with necrotizing scleritis. The patient was treated with systemic steroids and cyclophosphamide, which reduced the ocular pain but did not improve visual acuity, needing surgical treatment of the scleral coverage defect. While necrotizing scleritis is an unusual manifestation of EGPA, it should be part of the differential diagnosis in patients with red eye or ocular pain as an accurate diagnosis and prompt treatment could reduce local complications.

摘要

嗜酸性肉芽肿性多血管炎(EGPA)是一种与抗中性粒细胞胞浆抗体(ANCA)相关的血管炎,其特征为出现富含嗜酸性粒细胞的坏死性肉芽肿以及中小血管的血管炎,可累及呼吸道、周围神经系统,眼部受累较少见。我们报告一例54岁患有EGPA病史的白人男性,其出现眼痛、眼红、视力丧失,并有与坏死性巩膜炎相符的巩膜变薄迹象。该患者接受了全身用类固醇和环磷酰胺治疗,眼痛减轻但视力未改善,需要对巩膜覆盖缺损进行手术治疗。虽然坏死性巩膜炎是EGPA的一种不常见表现,但在出现眼红或眼痛的患者中应将其纳入鉴别诊断,因为准确诊断和及时治疗可减少局部并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f68/10009021/d1fb02c1d89c/10.1177_11795476231161167-fig1.jpg

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