Retroperitoneal fibrosis

Lancet. 2006 Jan 21;367(9506):241-51. doi: 10.1016/S0140-6736(06)68035-5.


Retroperitoneal fibrosis encompasses a range of diseases characterised by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighbouring structures--eg, ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Idiopathic disease was thought to result from a local inflammatory reaction to antigens in the atherosclerotic plaques of the abdominal aorta, but clinicolaboratory findings--namely, the presence of constitutional symptoms and the high concentrations of acute-phase reactants--and the frequent association of the disease with autoimmune diseases that involve other organs suggest that it might be a manifestation of a systemic autoimmune or inflammatory disease. Steroids are normally used to treat idiopathic retroperitoneal fibrosis, although other options--eg, immunosuppressants, tamoxifen--are available. The outlook is usually good, but, if not appropriately diagnosed or treated, the disease can cause severe complications, such as end-stage renal failure. Here, we review the different aspects of retroperitoneal fibrosis, focusing on idiopathic retroperitoneal fibrosis and on the differential diagnosis associated with the secondary forms.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Diagnosis, Differential
  • Humans
  • Magnetic Resonance Imaging
  • Retroperitoneal Fibrosis / diagnosis
  • Retroperitoneal Fibrosis / etiology
  • Retroperitoneal Fibrosis / pathology*
  • Tomography, X-Ray Computed


  • Adrenal Cortex Hormones