Department of Ophthalmology, University Hospital Virgen Macarena, Seville, Spain.
Members of RETICS OFTARED "RD16/0008/0010", Financed by the Instituto de Salud Carlos III, as Part of the Plan Nacional I+D+i, and Co-Financed by the European Union (FEDER/FSE) "Una manera de hacer Europa 2013-2016".
Ocul Immunol Inflamm. 2024 Jul;32(5):775-777. doi: 10.1080/09273948.2023.2191121. Epub 2023 Apr 6.
To report a case of isolated conjunctival inflammation as initial manifestation of IgG4-related disease and subsequent development of panuveitis.
A 75-year-old female presented with a diffuse mass lesion in the temporal area of the left eye, involving the conjunctiva, and an abscessed corneal ulcer. An incisional biopsy was diagnostic of IgG4-related disease with an elevated IgG4/IgG ratio (>40%) and the presence of >10 cells that tested positive for IgG4/CGA. No other ocular, orbital or systemic manifestations were noted at the time of diagnosis. After a year of treatment with topical dexamethasone, oral prednisone, and methotrexate, the patient developed panuveitis, which was controlled by increasing steroids and switching to rituximab.
IgG4-related disease is a rare entity that can be particularly challenging to diagnose if it manifests in an atypical manner. Continuous follow-up of patients is crucial as relapses and worsening of symptoms can occur despite treatment.
报告一例以孤立性结膜炎为首发表现，随后发展为全葡萄膜炎的 IgG4 相关疾病病例。
一名 75 岁女性因左眼颞部弥漫性肿块病变就诊，病变累及结膜并伴有角膜脓肿溃疡。经切开活检诊断为 IgG4 相关疾病，IgG4/IgG 比值升高（>40%），且有>10 个细胞 IgG4/CGA 阳性。在诊断时未发现其他眼部、眶部或全身表现。在接受局部地塞米松、口服泼尼松和甲氨蝶呤治疗一年后，患者出现全葡萄膜炎，经增加类固醇和改用利妥昔单抗治疗后得到控制。
IgG4 相关疾病较为罕见，如果表现不典型，诊断尤其具有挑战性。尽管进行了治疗，但患者仍有复发和症状恶化的可能，因此对患者进行持续随访至关重要。
