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IgG4-related retroperitoneal fibrosis

Disease definition

A rare systemic autoimmune disease characterized by mass-forming, potentially destructive inflammation and fibrosis in the soft tissues of the retroperitoneum, associated with elevation of serum IgG4 levels and infiltration of IgG4-positive plasma cells in at least one organ or site. Most frequent locations are peripheral to the abdominal aorta, as well as the iliac and renal arteries. Clinical symptoms are unspecific and include abdominal pain, back pain, and edema of the lower extremities. The condition may occur together with IgG4-related disease in other parts of the body.

ORPHA:49041

Classification level: Subtype of disorder
  • 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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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.