Retroperitoneal fibrosis is a well-characterized chronic inflammatory
process that leads to several other fibrosing diseases and may be part
of a systemic fibrosing disease. The vast majority of cases are idiop
athic, whereas the remainder are associated with methysergide ingestio
n, malignancy, and abdominal aortic aneurysms. An immune-mediated etio
logy has been proposed. The symptoms of retroperitoneal fibrosis are v
ague and nonspecific, making radiology the key to its diagnosis. Treat
ment aims to relieve symptoms and halt the progression of the disease.
This article reviews the pathogenesis, presentation, radiologic diagn
osis, and treatment of the disease.