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IgG4-related retroperitoneal fibrosis

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.

ORPHA:49041

  • Synonym(s):
    • Idiopathic retroperitoneal fibrosis
    • Ormond disease
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable or Unknown 
  • Age of onset: Adult
  • ICD-10: N13.5
  • OMIM: 228800
  • UMLS: C0035357
  • MeSH: D012185
  • GARD: 9568
  • MedDRA: 10038979

Summary

Epidemiology

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.

Etiology

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.

- Last update: May 2006

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The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.