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IgG4相关性疾病（ IgG4－RD）是一种新被认识的系统性、纤维炎症性疾病，该病以多位点形成肿瘤样脱髓鞘病变、大量IgG4阳性淋巴细胞和浆细胞浸润以及纤维化为特征，常累及胰腺、胆管、唾液腺、眶周组织、淋巴结及腹膜后间隙等。各器官的病理特征相似，诊断该病时，需结合临床表现、实验室、组织病理及影像学等检查结果，并积极排除其他疾病如肿瘤后作出诊断。该病对激素等免疫抑制剂治疗反应良好，预后较好。“,”IgG4-related disease(IgG4-RD) is a newly recognized systemic fibro-inflammatory disorder characterized with a tendency to form tumefactive lesions in multiple sites ,massive IgG4-bearing lymphocyte and plasma cell infiltration and fibrosis.The most commonly involved the pancreas,the biliary tree,salivary glands,periorbital tissues,lymph nodes,retroperitoneal space and so on.The histopathological features are similar across the involved organs.Combination of the clinical manifestations,laboratory examination,patholog-ic and imaging findings is recommended for diagnosis of IgG4-RD,while other disorders such as malignancy should be excluded to make a final diagnosis.Treatments with corticosteroid and other immunosuppressive drugs are effective and the prognosis is favorable.