Department of Ophthalmology, The Second Xiangya Hospital, Central South University, No. 139, Renmin Middle Road, Furong District, Changsha, 410011, China.
Hunan Clinical Research Center of Ophthalmic Disease, Changsha, China.
BMC Ophthalmol. 2023 Mar 9;23(1):92. doi: 10.1186/s12886-023-02822-7.
To report a rare case of IgG4-related ophthalmic disease (IgG4-ROD) manifesting as intraocular masses and scleritis in both eyes in a 61-year-old male and to investigate the changes in multimodal imaging features of the lesion sites and helper T-cell type 1 (Th 1)/Th 2/Th 17 cytokine levels in the aqueous humor.
A patient with IgG4-ROD seemingly manifested with an intraocular tumor in the left eye and sequentially, with an inflammatory mass in the ciliary body and scleritis in the right eye. The patient complained of vision loss of 6 months duration in the left eye at his first visit. With a preliminary diagnosis of an intraocular tumor, enucleation of the left eyeball and histopathological examination were performed. Approximately 3 months later, the patient started to experience headache, eye pain, and declining vision in the right eye. Ophthalmic imaging revealed a ciliary mass and scleritis. Th 1/Th 2/Th 17 cytokine levels and multimodal imaging findings were analyzed before and after corticosteroid treatment. Histopathological examination and immunohistochemistry (IHC) of the enucleated left eye demonstrated lymphoplasmacytic infiltration with an IgG4+/IgG+ cell ratio of approximately 40%, pointing to the diagnosis of probable IgG4-ROD. Long-term treatment with corticosteroids led to significant improvement in the signs and symptoms of the left eye. Th 1/Th 2/Th 17 cytokine profile monitoring of the aqueous humor and multimodal imaging of the right eye showed gradual regression of the mass and attenuation of ocular inflammation during treatment.
Patients with an atypical presentation of IgG4-ROD, such as intraocular masses and scleritis, are likely to experience a significant delay in diagnosis. This case demonstrates the significance of IgG4-ROD in the differential diagnosis of intraocular tumors and ocular inflammation. IgG4-RD is a newly diagnosed disease with multi-organ involvement and little is known about its pathogenesis, particularly in the eye. The present case will open new challenges in the clinico-pathological diagnosis and research of this disease. Combined investigations of multimodal imaging and cytokine level detection of intraocular fluid provide a new and effective way to monitor disease progression.
报道一例罕见的 IgG4 相关眼病（IgG4-ROD）病例，该病例表现为 61 岁男性双眼眼内肿块和巩膜炎，并探讨病变部位多模态影像学特征及房水辅助性 T 细胞 1（Th1）/Th2/Th17 细胞因子水平的变化。
患者 IgG4-ROD 似乎表现为左眼眼内肿瘤，随后右眼睫状体炎症性肿块和巩膜炎。患者首次就诊时左眼视力丧失 6 个月，初步诊断为眼内肿瘤，行左眼眼球摘除术和组织病理学检查。大约 3 个月后，患者开始出现右眼头痛、眼痛和视力下降。眼部影像学检查显示睫状体肿块和巩膜炎。分析了皮质类固醇治疗前后 Th1/Th2/Th17 细胞因子水平和多模态成像结果。对左眼眼球摘除物进行组织病理学检查和免疫组织化学（IHC）检查，结果显示淋巴浆细胞浸润，IgG4+/IgG+细胞比例约为 40%，提示可能为 IgG4-ROD。长期皮质类固醇治疗显著改善了左眼的症状和体征。右眼房水 Th1/Th2/Th17 细胞因子谱监测和多模态成像显示，治疗过程中肿块逐渐消退，眼部炎症减轻。
表现不典型的 IgG4-ROD 患者，如眼内肿块和巩膜炎，可能会显著延迟诊断。本例表明 IgG4-ROD 在眼内肿瘤和眼内炎症的鉴别诊断中具有重要意义。IgG4-RD 是一种新诊断的多器官受累疾病，其发病机制知之甚少，尤其是在眼部。本病例将为该病的临床病理诊断和研究带来新的挑战。多模态成像和眼内液细胞因子水平检测的联合研究为监测疾病进展提供了一种新的有效方法。
