Barut Selver Ozlem, Ahmadli Emil, Tas Muhammed Dara, Yaman Banu, Ceylan Naim, Kanavi Mozhgan Rezaei
Department of Ophthalmology, Ege University Faculty of Medicine, Izmir, Turkey.
Ocular Surface Research Laboratory, Ege University, Faculty of Medicine, Izmir, Turkey.
J Ophthalmic Vis Res. 2025 Aug 27;20. doi: 10.18502/jovr.v20.16399. eCollection 2025.
To report a case of eosinophilic granulomatosis with polyangiitis (EGPA) initially presenting as orbital involvement, describe its successful management, and provide a comprehensive literature review.
A 33-year-old female patient presented with swelling, redness, tenderness, and a mass under the left upper eyelid for one month. Upper lid eversion showed a multilobulated lesion in the subconjunctival area of the same region. The patient's medical history included asthma and atrial septal defect surgery. Orbital MRI revealed a soft tissue mass infiltrating the superior and lateral aspects of the conal and extraconal regions in the anterior orbit, with extension toward the preseptal area. The lesion underwent incisional biopsy, and histopathological findings were consistent with the diagnosis of EGPA. The patient's blood tests revealed eosinophilia and a negative antineutrophil cytoplasmic antibody. After excluding other similar pathologies such as granulomatosis with polyangiitis, we observed a dramatic regression in her orbital lesion following systemic steroid therapy.
The diagnosis of EGPA, a rare clinical presentation, is crucial for ophthalmologists because it provides early recognition of the systemic disease and can help slow its progression by initiating appropriate treatment in a timely manner.
报告一例最初表现为眼眶受累的嗜酸性肉芽肿性多血管炎（EGPA）病例，描述其成功的治疗过程，并提供全面的文献综述。
一名33岁女性患者，左上眼睑肿胀、发红、压痛伴肿物1个月。上睑翻转显示同一区域结膜下有一个多叶状病变。患者既往病史包括哮喘和房间隔缺损手术史。眼眶MRI显示前眼眶圆锥内和圆锥外区域的上外侧有一个软组织肿块浸润，并延伸至眶隔前区域。该病变进行了切开活检，组织病理学结果符合EGPA诊断。患者血液检查显示嗜酸性粒细胞增多，抗中性粒细胞胞浆抗体阴性。在排除其他类似疾病如肉芽肿性多血管炎后，我们观察到全身类固醇治疗后其眼眶病变显著消退。
EGPA这种罕见的临床表现的诊断对眼科医生至关重要，因为它能使全身疾病得到早期识别，并通过及时启动适当治疗有助于减缓其进展。
