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Retroperitoneal fibrosis.

Augusto Vaglio1, Carlo Salvarani, Carlo Buzio

  • 1Department of Clinical Medicine, Nephrology and Health Science, University of Parma, Via Gramsci 14, 43100 Parma, Italy. augusto.vaglio@virgilio.it

Lancet (London, England)
|January 24, 2006
PubMed
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Retroperitoneal fibrosis is a condition involving inflammation and tissue growth around major abdominal vessels. While often idiopathic, it can be systemic and requires timely diagnosis and treatment to prevent complications like kidney failure.

Area of Science:

  • Nephrology
  • Rheumatology
  • Vascular Surgery

Background:

  • Retroperitoneal fibrosis (RPF) is characterized by fibro-inflammatory tissue encasing retroperitoneal structures, commonly the aorta and iliac arteries.
  • While often idiopathic, RPF can be secondary to drugs, malignancy, infection, or surgery.
  • Emerging evidence suggests idiopathic RPF may stem from a systemic autoimmune or inflammatory process, not just localized aortic atherosclerosis.

Purpose of the Study:

  • To review the multifaceted aspects of retroperitoneal fibrosis.
  • To focus on idiopathic retroperitoneal fibrosis (iRPF).
  • To discuss the differential diagnosis of secondary forms of RPF.

Main Methods:

  • Review of existing literature on retroperitoneal fibrosis.

Related Experiment Videos

  • Analysis of clinicolaboratory findings associated with iRPF.
  • Comparison of idiopathic and secondary RPF presentations and etiologies.

    • Main Results:

    • Idiopathic RPF presents with constitutional symptoms and elevated acute-phase reactants, suggesting a systemic inflammatory basis.
    • RPF can lead to severe complications, including end-stage renal failure, if not managed promptly.
    • Steroids are a primary treatment for iRPF, with immunosuppressants and tamoxifen as alternatives.

    Conclusions:

    • Retroperitoneal fibrosis is a complex condition with both idiopathic and secondary causes.
    • Systemic autoimmune or inflammatory processes are implicated in idiopathic RPF.
    • Accurate diagnosis and treatment are crucial to avert severe renal complications.