Liu Jia, Zhang Bei
Department of Ophthalmology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, 3 Qingchun East Road, Hangzhou, 310016, China.
Key Laboratory for Corneal Diseases Research of Zhejiang Province, 3 Qingchun East Road, Hangzhou, 310016, China.
BMC Ophthalmol. 2025 Apr 9;25(1):187. doi: 10.1186/s12886-025-04031-w.
To report a case of bilateral corneoscleritis with retrocorneal plaque lesion as the presenting sign of granulomatous polyangiitis (GPA).
A 61-year-old female presented with bilateral extensive periphery corneoscleritis with retrocorneal plaque and prominent conjunctival injection. She suffered from progressively blurry vision and pain in both eyes for about 5 years, accompanied by sinusitis. No retinal or orbital abnormalities were noted on examination. A conjunctival and scleral biopsy was performed in the left eye, and the histopathological analysis was consistent with granulomatous inflammation. A full diagnostic workup revealed no obvious abnormal findings except positive cytoplasmic antineutrophil cytoplasmic antibody (cANCA) and proteinase 3-specific antineutrophil cytoplasmic antibody (PR3-ANCA). The patient achieved disease remission with cyclophosphamide and corticosteroid and remained on oral and topical medications with no further loss of vision or discomfort.
This is a unique documented case of corneoscleritis with retrocorneal plaque lesion as the initial presenting feature of GPA. Our case illustrates that the initial ocular manifestations of GPA involving the cornea and sclera may have atypical lesions. Clinicians should cautiously evaluate the patients to enhance the early diagnosis and promptly initiate treatment measures to prevent vision loss.
报告1例以角膜后斑块病变为首发表现的肉芽肿性多血管炎（GPA）伴双侧角膜巩膜炎。
一名61岁女性，表现为双侧广泛性周边角膜巩膜炎伴角膜后斑块及明显的结膜充血。她双眼渐进性视力模糊和疼痛约5年，伴有鼻窦炎。检查未发现视网膜或眼眶异常。对左眼进行了结膜和巩膜活检，组织病理学分析符合肉芽肿性炎症。全面的诊断检查除了胞浆抗中性粒细胞胞浆抗体（cANCA）和蛋白酶3特异性抗中性粒细胞胞浆抗体（PR3-ANCA）阳性外，未发现明显异常。患者通过环磷酰胺和皮质类固醇实现疾病缓解，并继续接受口服和局部用药，未再出现视力丧失或不适。
这是1例有文献记载的独特病例，以角膜后斑块病变为GPA的初始表现特征。我们的病例表明，GPA累及角膜和巩膜的初始眼部表现可能有非典型病变。临床医生应谨慎评估患者，以加强早期诊断并及时启动治疗措施，防止视力丧失。
