Retroperitoneal fibrosis. A true connective tissue disease

Rheum Dis Clin North Am. 1996 Feb;22(1):23-38. doi: 10.1016/s0889-857x(05)70260-x.


Retroperitoneal fibrosis, a disease that can occur at any age, is characterized by a periaortic fibrous mass that often surrounds the ureters, leading to ureteral obstruction. Patients who present with this disease may complain of flank pain and acute renal failure. There is a high correlation with atherosclerotic disease of the aorta, although the pathogenesis of this disease remains unknown. Although recommendations for therapy remain empiric, prednisone seems to be efficacious in treatment; tamoxifen also may be effective. Retroperitoneal fibrosis usually does not lead to long-term morbidity or affect survival.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Prognosis
  • Retroperitoneal Fibrosis / complications
  • Retroperitoneal Fibrosis / epidemiology
  • Retroperitoneal Fibrosis / pathology*
  • Retroperitoneal Fibrosis / therapy