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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

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