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IgG4-related retroperitoneal fibrosis

Synonym(s) Idiopathic retroperitoneal fibrosis
Ormond disease
Prevalence 1-9 / 100 000
Inheritance Not applicable
or Unknown
Age of onset Adult
  • N13.5
  • C0035357
  • D012185
  • 10038979


Disease definition

Retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding retroperitoneal structures, such as aorta, vena cava, ureters and psoas muscle.


Its incidence varies between 0.4 and 1/200 000.

Clinical description

Symptoms may vary depending upon the location and extent of fibrotic growth. The commonest manifestations are pain, weight loss, nausea and vomiting. In the most severe cases, the fibrotic process can lead to ureteric obstruction and renal failure. Retroperitoneal fibrosis is an unusual cause of obstructive uropathy among children.


Aetiology is unknown in most patients, hence the name ``idiopathic retroperitoneal fibrosis''.

Diagnostic methods

RPF can be diagnosed by computed tomography, magnetic resonance imaging or biopsy. An early diagnosis helps to preserve renal function and avoid involvement of other organs.

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Detailed information

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