特发性腹膜后纤维化（IRPF）是一种罕见的纤维炎症性疾病，其发病可能与自身免疫有关。IRPF主要累及腹主动脉、髂动脉、输尿管及其邻近的腹膜后组织，目前也被认为是免疫球蛋白G4（IgG4）相关性疾病的一种。因病变累及多种器官，故临床表现复杂多变，除了非特异性表现如腹痛、乏力、体重降低外，泌尿系梗阻是其最常见的并发症。腹膜后纤维化（RPF）的临床诊断主要依靠电子计算机断层扫描（CT）和磁共振成像（MRI）检查，病理活组织检查可以确诊，但由于取材困难，其必要性仍存争议。超声可用于RPF的相关并发症的发现及随访，正电子发射计算机断层扫描也是疾病活动性评估及随访重要手段，作者对该病临床诊治进展作一综述。

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