目的：研究IgG4相关性眼病(IgG4-related ophthalmic disease, IgG4-ROD)患者的影像学特征与外周血免疫球蛋白G4(IgG4)水平之间的相关性，为评估IgG4相关性疾病全身性严重程度提供新思路。方法：收集2023年8月—2024年9月在吉林大学第二医院眼科医院经术后组织标本病理确诊的29例IgG4-ROD阳性患者。回顾性分析患者眼眶影像学特点与血清IgG4水平相关性，探讨影像学中特征性表现包括泪腺肿大、三叉神经分支增粗、眼外肌增粗、鼻黏膜类炎症改变、眼睑软组织肥厚，以及其他眶内软组织增生等特征性影像学改变出现比例，并按照累及组织结构情况分级评分，评估特征性影像学改变与血清IgG4水平之间的相关性。结果：29例病理确诊IgG4-ROD患者中，泪腺均受累，占比100%；眼外肌受累17例，占比58.62%；三叉神经分支受累5例(4例眶下神经受累，3例额神经病受累，2例眶下神经与额神经同时受累)，占比17.24%眼睑软组织肥厚24例，占比82.76%鼻黏膜出现类炎症反应15例，占比51.72%；合并眶内其他软组织增生性病变2例，占比6.90%。影像学中特征性受累组织结构分级评分与血清IgG4水平呈正相关(P < 0.05)。结论：IgG4-ROD影像学中特征性组织结构受累及范围与血清IgG4水平明显相关，可以辅助评估IgG4相关性疾病全身性严重程度。

Objective: To investigate the correlation between the imaging characteristics of patients with IgG4-related ophthalmic disease (IgG4-ROD) and the serum immunoglobulin G4 (IgG4) levels, providing new insights for assessing the systemic severity of IgG4-related diseases. Methods: This study collected postoperative tissue samples from 29 patients with histopathologically conffrmed IgG4-ROD at the Ophthalmology Department of Jilin University Second Hospital from August 2023 to September 2024. TTis study retrospectively analyzed the correlation between patients' orbital imaging features and serum IgG4 levels, and explored the proportion of characteristic imaging changes including enlargement of the lacrimal gland, thickening of the trigeminal nerve branches, thickening of the extraocular muscles, inffammatory like changes of the nasal mucous membranes, hypertrophy of the eyelid soft tissues, as well as hyperplasia of other intraorbital soft tissues in the imaging. A grading score for affected tissue structures was established to evaluate the correlation between characteristic imaging changes and serum IgG4 levels. Results: Among the 29 patients diagnosed with IgG4-ROD, lacrimal gland involvement was observed in all patients (100%). Extraocular muscle involvement was present in 17 patients (58.62%). Five patients had involvement of the trigeminal nerve branches (including 4 with infraorbital nerve involvement and 3 with frontal nerve involvement, with 2 patients having simultaneous involvement of both nerves), accounting for 17.24% of the cases. Eyelid soff tissue hypertrophy was observed in 24 patients (82.76%), and nasal mucosal inflammatory responses were noted in 15 patients (51.72%). Additionally, two patients (6.90%) presented with other proliferative lesions within the orbit. The correlation analysis between the grading scores for imaging features and serum IgG4 levels demonstrated a significant positive correlation. Conclusions: The extent of characteristic structural involvement observed in the imaging features of IgG4-ROD is significantly correlated with serum IgG4 levels. TTis correlation can assist in evaluating the systemic severity of IgG4-related diseases and provides clinical evidence supporting the need for comprehensive systemic evaluations, such as PET-CT, in patients whose initial presentation is IgG4-related ophthalmic disease.