IgG4-related retroperitoneal fibrosis

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Synonyms: Idiopathic retroperitoneal fibrosis | Ormond disease

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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version July 2024

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IgG4-related retroperitoneal fibrosis?

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