Berkowitz Eran, Arnon Ella, Yaakobi Alona, Cohen Yuval, Tiosano Beatrice
Department of Ophthalmology, Hillel Yaffe Medical Center Affiliated with The Bruce Rappaport School of Medicine, The Technion, Haifa, Israel.
Case Rep Ophthalmol Med. 2017;2017:4876587. doi: 10.1155/2017/4876587. Epub 2017 Jan 9.
A rare case of IgG4-related disease (IgG4-RD) manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms. This case highlights the significance of IgG4-RD in the differential diagnosis of scleritis and raises the question as to whether various organs affected by IgG4-RD may have different underlying pathophysiological mechanisms in which pathogenic T cells play a role.
本文报告了一例20岁女性表现为结节性巩膜炎的罕见IgG4相关疾病（IgG4-RD）病例。患者主诉左眼疼痛、发红一周。眼部检查及辅助检查诊断为结节性巩膜炎。患者在接受一周全身类固醇治疗后未出现明显改善，遂对病变区域进行活检，显示出IgG4-RD的组织病理学特征。长期使用皮质类固醇和一种类固醇节约剂（甲氨蝶呤）治疗后，体征和症状有显著改善。该病例突出了IgG4-RD在巩膜炎鉴别诊断中的重要性，并提出了受IgG4-RD影响的各个器官是否可能具有不同的潜在病理生理机制（其中致病性T细胞起作用）的问题。
