IgG4相关性眼病（immunoglobulin G4-related ophthalmic disease, IgG4-ROD）是一种与IgG4阳性浆细胞浸润和血清IgG4水平升高相关的系统性疾病。该病的眼部表现包括泪腺、眼眶脂肪、眶下神经、眼外肌和眼睑的受累，且常伴有炎症和纤维化过程。在分子水平上，IgG4-ROD涉及多种免疫细胞和细胞因子的相互作用，包括辅助性T细胞1（T helper cell 1, Th1）、辅助性T细胞2（T helper cell 2, Th2）、调节性T细胞 (regulatory T cells, Tregs）和滤泡辅助性T细胞（follicular helper T cell, Tfh），以及由它们分泌的白细胞介素4（interleukin 4, IL-4）、白细胞介素13（interleukin 13, IL-13）和转化生长因子β（transforming growth factor-β,  TGF-β）等。这些分子通过促进B细胞活化和IgG4的产生，以及纤维化过程的发生，共同参与了IgG4-ROD的发病机制。诊断依赖于组织病理学特征，治疗通常包括糖皮质激素和免疫抑制剂，旨在控制免疫介导的炎症和纤维化，减轻症状并防止器官损伤。随着对IgG4-ROD在分子层面发病机制认识的深入，治疗策略将不断优化，进而为患者提供更为精准和有效的治疗方案，从而改善患者预后，提高患者生活质量。本文基于现有研究，总结并阐述了与IgG4-ROD致病有关的关键分子及信号通路，从分子视角对IgG4-ROD的自身免疫、纤维化及二者之间的联系与转变进行综述。

Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) is a systemic disorder characterized by the infiltration of IgG4-positive plasma cells and elevated serum IgG4 levels. The ocular symptoms of this disease can affect multiple structures, including the lacrimal gland, orbital fat, infraorbital nerves, extraocular muscles, and eyelids.These manifestations are often accompanied by inflammatory and fibrotic processes. At the molecular level, IgG4-ROD is linked to the interaction of various immune cells and cytokines. These include T helper cell 1 (Th1), T helper cell 2 (Th2), regulatory T cells (Tregs), and follicular helper T cells (Tfh), along with cytokines interleukin 4 (IL-4), interleukin 13 (IL-13), and transforming growth factor-β (TGF-β). They promote B cell activation and IgG4 production, and also facilitate the development of fibrosis. Diagnosis of IgG4-ROD is mainly based on histopathological features. Treatment typically involves the use of glucocorticoids and immunosuppressive agents, which aim to control immune-mediated inflammation and fibrosis, alleviate symptoms, and prevent organ damage. As our understanding of the molecular pathogenesis of IgG4-ROD advances, it is anticipated that treatment strategies will be continuously refined. This will enable us to offer patients more precise and effective therapeutic options, ultimately improving prognosis and quality of life. This article provides a summary and clarification of the key molecules and signaling pathways involved in the pathogenesis of IgG4-ROD. It also reviews,   from a molecular perspective, the interplay between autoimmunity and fibrosis, as well as their transformations.

年龄相关性黄斑变性(age-related macular degeneration,AMD)是一种发生在黄斑区的退行性变，其中湿性年龄相关性黄斑变性(wet age-related macular degeneration,wAMD)以黄斑区新生血管为主要病理特征，是导致老年人视力受损甚至失明的重要原因，视网膜下纤维化是wAMD最常见的自然后遗症，可导致光感受器、视网膜色素上皮(retinal pigment epithelial,RPE)和脉络膜毛细血管受损，导致不可逆转的中心视力丧失。多种基线特征被发现是视网膜下纤维化的危险因素，可用于预测早期视网膜下纤维化的发生。迄今为止，还没有有效的抗纤维化治疗方法，抗血管内皮生长因子(anti-vascular endothelia growth factor, anti-VEGF)治疗是wAMD的一线治疗方案，该治疗方法不能改善视网膜下纤维化，但及时启动治疗可能有助于预防或延缓纤维化的进展，目前多种靶向分子药物正被研发用于抗纤维化的治疗。该文综述了wAMD视网膜下纤维化的临床表现及意义、预测纤维化形成的基线特征、基本发病机制及潜在的抗纤维化治疗方法，旨在为临床诊治工作提供参考。

Age-related macular degeneration (AMD) is a degenerative disease of the macular, and wet age-related macular degeneration(wAMD) is mainly characterized by macular neovascularization, which is an important reason of visual impairment or even blindness in the elderly. Subretinal fibrosis is the most common natural sequelae of wAMD, which can lead to irreversible central vision loss by damaging photoreceptors, RPE, and choroidal capillaries. Multiple baseline features have been identified as the risk factors for subretinal fibrosis, which can be used to predict the early subretinal fibrosis. Heretofore, no anti fibrotic treatment method is effective. Anti vascular endothelial growth factor (anti VEGF) treatment is the first-line treatment for wAMD. This therapy cannot improve subretinal fibrosis, but timely initiation of treatment may help prevent or delay the progression of fibrosis. Currently, multiple targeted molecular drugs are being developed for anti fibrotic treatment. This article reviews the clinical manifestations and significance of subretinal fibrosis in wet age-related macular degeneration, baseline features for predicting the formation of fibrosis, basic pathogenesis, and potential anti-fibrosis treatment methods,aiming to provide reference for clinical diagnosis and treatment.