Abstract

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs.RPF can be divided into two types: idiopathic and secondary.Some of the previously diagnosed idiopathic retroperitoneal fibrosis cases belong to the IgG4-related disease category.In order to help clinicians to achieve better diagnostic accuracy, the article reviews current national and international research on this disorder, and summarizes the epidemiological characteristics, the pathogenesis, the clinical manifestations, the microscopicand imaging features, and the most recent advances in the management of RPF. Key words: Retroperitoneal Fibrosis; Idiopathic retroperitoneal fibrosis; IgG4 related disease

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