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Retroperitoneal Fibrosis (RPF, Ormond Disease) Updated 4/2010

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BASICS

  • Description
  • Epidemiology
  • Risk Factors
  • General Prevention
  • Pathophysiology
  • Associated Conditions

DIAGNOSIS

  • Signs and Symptoms
  • Tests
  • Differential Diagnosis

TREATMENT

  • Medication (Drugs)
  • Surgery
  • Additional Treatment | Radiotherapy

Ongoing Care

  • Prognosis
  • Complications
  • Follow-Up Recommendations
The following is an excerpt....
BASICS
Description
  • RPF is characterized by sclerotic tissue from inflammatory processes causing encasement of the retroperitoneal structures including the ureters, aorta and IVC. The main manifestation is obstructive uropathy.
  • Exhibits a perivascular distribution, typically including the periaortic, pericaval, and peri-iliac retroperitoneum.
  • Generally classified as either primary (idiopathic) or secondary RPF
  • Hallmark is medical deviation of the ureters on imaging with or without hydronephrosis.
Epidemiology
  • Unknown, but estimated at 1:200,000–1:500,000/yr
  • In Finland, incidence is 0.1:100,000/yr
Risk Factors
  • Asbestos exposure
  • Associated with autoimmune disorders
  • Abdominal aortic aneurysm
  • Male > Female (2–3:1)
  • RPF most common in the 5th–6th decades, but can occur at any age
  • Use of implicated medications (see ...

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