IgG4相关性疾病(IgG4-RD)是一种系统性炎症纤维化疾病,几乎可影响每一个器官系统,包括胰腺、胆道、主动脉、肺、唾液腺和泪腺、甲状腺、硬脊膜和肾脏等。在过去15年中发现其疾病特点是肿瘤样病变和两个标志性的组织学特征,即 IgG4-related disease (IgG4-RD) is a systemic inflammatory fibrosis disease affecting almost every organ system, including the pancreas, biliary tract, aorta, lung, salivary and lacrimal glands, thyroid, dura and kidney. In the past 15 years, the disease has been characterized by tumor-like lesions and two hallmarks of histological features, namely