Pitz Susanne
Bürgerhospital, Augenklinik - Orbitazentrum, Nibelungenallee 37-41, 60318, Frankfurt, Deutschland.
Ophthalmologe. 2021 Aug;118(8):787-793. doi: 10.1007/s00347-021-01448-8. Epub 2021 Jul 1.
The IgG4-related systemic disease as well as the homonymous variant IgG4-related orbital disease were first described less than 15 years ago. The mostly subacute clinical symptoms can be multifarious and the classical case is characterized by an orbital inflammatory condition with a bilateral enlargement of the lacrimal glands; however, any other orbital tissue with the exception of the eyeball can be affected by the lymphocytic inflammatory infiltration.
Based on the current literature the clinical picture, epidemiology, pathogenesis and treatment options are described. A focus is on the differential diagnostic demarcation from other inflammatory processes of the orbit.
The IgG4-related orbital disease is an important differential diagnosis of inflammatory diseases of the orbit. The condition can exhibit considerable clinical and imaging similarity to idiopathic inflammation of the orbit, to the specific inflammations seen in systemic diseases, to Graves' orbitopathy and to lymphoproliferative diseases and lymphoma. After histopathologic confirmation the interdisciplinary clarification and treatment consensus are indispensable.
IgG4相关性全身性疾病以及同名的IgG4相关性眼眶疾病在不到15年前首次被描述。其临床症状大多为亚急性，表现多样，典型病例以眼眶炎症状态伴双侧泪腺肿大为特征；然而，除眼球外的任何其他眼眶组织均可受到淋巴细胞炎性浸润的影响。
基于当前文献描述了其临床表现、流行病学、发病机制及治疗选择。重点在于与眼眶其他炎症过程的鉴别诊断。
IgG4相关性眼眶疾病是眼眶炎症性疾病的重要鉴别诊断。该疾病在临床和影像学上可能与眼眶特发性炎症、全身性疾病中的特定炎症、格雷夫斯眼病以及淋巴增殖性疾病和淋巴瘤有显著相似性。组织病理学确诊后，跨学科的明确诊断和治疗共识必不可少。
