Korean J Nephrol.  2007 Mar;26(2):269-273.

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

• Koo JM
• Kim YH
• Hong HJ
• Kim HI
• Yoon SK
• An WS
• Lee SW
• Chung WT

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.

Keyword

Retroperitoneal fibrosis; Recurrence; Steroids; Tamoxifen

MeSH Terms

Autoimmune Diseases
Diagnosis
Hydronephrosis
Immunosuppressive Agents
Kidney Failure, Chronic
Prognosis
Rare Diseases
Recurrence
Retroperitoneal Fibrosis*
Steroids*
Tamoxifen*
Ureter
Immunosuppressive Agents
Steroids
Tamoxifen