IgG4相关性疾病的研究进展

IgG4-related diseases: A comprehensive review

IgG4相关性疾病(IgG4-RD)是多器官受累缓慢进展的炎症性疾病,以受累器官内出现大量IgG4阳性浆细胞浸润及纤维化为特征,其具体机制不清且涉及较广泛。当前,获得性免疫和适应性免疫的异常调节被认为是IgG4-RD的主要发病机制,临床表现多种多样, IgG4-RD可累及任何组织器官,以炎性假瘤和弥漫性肿大为常见表现,易误诊为肿瘤或炎症。以糖皮质激素为主的药物治疗效果良好,但IgG4-RD症状多变,预后多不良。我们主要总结了IgG4-RD的发病机制、临床特征、诊断和治疗的相关研究进展。

IgG4-related disease(IgG4-RD)is an inflammatory disorder with slow progression in multiple organs,characterized by abundant infiltration of IgG4-positive plasmacytes and fibrosis in the involved organs.The precise pathogenic mechanism of IgG4-RD still remains unclear.Currently,the abnormal regulation of acquired immunity and adaptive immunity is considered as the main pathogenesis of IgG4-RD,and its clinical manifestations are diverse.IgG4-RD can affect any tissue and organ.Inflammatory pseudotumor and diffuse enlargement are the common manifestations,which are easily misdiagnosed as tumor or inflammation.The treatment of glucocorticoid-based drugs is effective.However,the symptoms of IgG4-RD are variable and the prognosis is poor.We mainly summarized the research progress in the pathogenesis,clinical features,diagnosis and treatment of IgG4-RD.