Abstract：
We present a case of an uncommon presentation of IgG4-related ophthalmic disease (ROD). A 58-year-old female presented with unilateral acute anterior uveitis of the right eye, which progressed to scleritis with the development of an associated orbital mass despite treatment with oral glucocorticoid. Initial histopathology of an orbital biopsy was non-diagnostic and continued progression of the disease lead to complete loss of vision in the right eye. The development of uveitis in the previously unaffected left eye led to the decision for enucleation of the right globe and further orbital biopsy. Histopathology revealed features supporting IgG4-related ophthalmic disease. Oral glucocorticoid therapy failed to induce remission, and rituximab therapy was initiated, leading to a rapid resolution in her symptoms. Other cases with a similar presentation report a poor visual prognosis, highlighting the need for prompt diagnosis and treatment of uveitis associated with signs of orbital or scleral involvement.
摘要：
我们介绍了一例罕见的IgG4相关眼科疾病（ROD）。一名58岁女性出现单侧急性右眼前葡萄膜炎，尽管口服糖皮质激素治疗，但进展为巩膜炎，并伴有眼眶肿块。眼眶活检的初始组织病理学无法诊断，并且疾病的持续进展导致右眼完全丧失视力。先前未受影响的左眼葡萄膜炎的发展导致决定摘除右眼球和进一步的眼眶活检。组织病理学显示支持IgG4相关眼科疾病的特征。口服糖皮质激素治疗未能引起缓解，利妥昔单抗开始治疗，导致她的症状迅速缓解。其他具有类似表现的病例报告视力预后不良，强调需要及时诊断和治疗与眼眶或巩膜受累迹象相关的葡萄膜炎。
