A Case of Recurred Retroperitoneal Fibrosis Treated by Combination Therapy of Steroids and Tamoxifen / 대한신장학회지

ABSTRACT

Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by inflammatory and fibrous tissue proliferation in the retroperitoneum, which often surrounds the adjacent retroperitoneal structure to cause compressive complications such as hydronephrosis. Its pathogenesis is still unknown, but recently the association with autoimmune disease is being suggested. Although the prognosis is generally good, if diagnosis or treatment is delayed, the disease can progress to acute or chronic renal failure. In the past, surgical procedures such as ureterolysis or transpositioning of the involved ureter were the mainstay of the treatment. But recent studies using steroids, immunosuppressants and tamoxifen have reported generally good results. However, the adequate dose or duration of medical treatment and the treatment for recurred disease have not been established yet. We report a case of that retroperitoneal fibrosis recurred 1 year after 2-weeks steroids therapy. It was successfully treated with combination therapy of steroids and tamoxifen.