Abstract：
Idiopathic retroperitoneal fibrosis (iRPF) is a chronic autoimmune disease characterized by fibroinflammatory tissue surrounding the abdominal aorta and iliac arteries and extending into the retroperitoneum to envelop neighboring structures. Hydronephrosis due to obstruction of ureters is the most common complication of iRPF. Glucocorticoid with or without immunosuppressants or tamoxifen, the mainstay of iRPF treatment, usually brings good response. Nevertheless, in some conditions, the obstruction of ureters remains unresolved with the treatment of all these medications. One of the reasons lies in the innate feature of the fibroinflammatory tissue. The proliferation of fibrosis tissue in addition to inflammation in the mass was associated with insufficient response to immunosuppressive therapies. Pirfenidone, an anti-fibrosis agent, has been successful in treating pulmonary fibrosis and renal fibrosis. Therefore, it is rationale to assume the effectiveness of pirfenidone in the treatment of iRPF. In the current article, we report a 61-year-old Chinese man with iRPF who responded well to pirfenidone.
摘要：
特发性腹膜后纤维化(iRPF)是一种慢性自身免疫性疾病,其特征在于围绕腹主动脉和髂动脉的纤维炎性组织并延伸到腹膜后以包裹邻近结构。输尿管阻塞引起的肾积水是iRPF最常见的并发症。糖皮质激素有或没有免疫抑制剂或他莫昔芬，iRPF治疗的支柱，通常会带来良好的反应。然而，在某些情况下,所有这些药物的治疗仍未解决输尿管阻塞。原因之一在于纤维炎症组织的先天特征。除肿块中的炎症外，纤维化组织的增殖与对免疫抑制疗法的反应不足有关。吡非尼酮,一种抗纤维化药物,已成功治疗肺纤维化和肾纤维化。因此,假设吡非尼酮治疗iRPF的有效性是合理的.在当前的文章中，我们报道了一名61岁的中国男性iRPF患者，他对吡非尼酮反应良好。
