Idiopathic retroperitoneal fibrosis (IRF) is a rare and unusual etiology of abdominal pain that is becoming more frequently diagnosed with the use of CT imaging for abdominal pain. Retroperitoneal fibrosis is characterized by the development of extensive fibrosis throughout the retroperitoneum that can encircle the aorta, iliac arteries, and ureters. About two thirds of retroperitoneal fibrosis cases are idiopathic, thought to be due to an autoimmune process of antibodies stimulating desmoplastic reaction. One third of cases are secondary to drugs (methysergide, beta-blockers, hydralazine, ergotamine, LSD) or diseases the stimulate desmoplastic reaction (tumors, infections, radiation, and Erdheim-Chester disease). Histology of IRF shows abundant fibrosis and chronic inflammation. There is limited data in the literature regarding idiopathic retroperitoneal fibrosis.
Derenge, MD, Candace
"Idiopathic Retroperitoneal Fibrosis,"
The Medicine Forum: Vol. 22, Article 17.
Available at: https://jdc.jefferson.edu/tmf/vol22/iss1/17