[IgG4-related diseases: A comprehensive review]

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.