IgG4相关性疾病的诊疗进展

IgG4 相关性疾病是一种与IgG4 淋巴细胞密切相关的慢性、系统性疾病，该类疾病以血清IgG4 水平升高以及IgG4 阳性细胞浸润多种器官和组织为特征，病理学改变呈现为席纹状纤维化，累及的器官或组织由于慢性炎症及纤维化进程可导致弥漫性肿大，常见受累器官包括泪腺、胰腺和腹膜后间隙等。该类疾病对皮质激素治疗反应良好。文中我们回顾了IgG4 相关性疾病前阶段的诊断标准，并补充不常见受累器官的国际诊断标准，更新各个诊断标准的国际共识，认为免疫抑制剂、生物制剂将成为治疗的新进展。

Progress in diagnosis and treatment of IgG4-related disease

IgG4-related disease is a kind of chronic and systemic disease which is characterized by increased serum level of IgG4 and abundant IgG4-bearing plasma cell infiltration, storiform fibrosis pathological features in the involved organs.Affected organs or tissues may show a diffuse swelling as a result of chronic inflammatory process, accompanied with progressive fibrosis. The most commonly involved organs or tissues include the salivary glands, pancreas, and retroperitoneal space and so on. A good response to glucocorticoid treatment. In this article, we reviewed before stage diagnostic standards about IgG4-related disease, add international diagnostic standards of uncommon affected organs，update diagnostic standards of international consensus，think the immune inhibitors and biological agents will become the new progress of treatment.