Home Education AUAUniversity Education Products & Resources Pathology for Urologists Retroperitoneum Idiopathic Retroperitoneal Fibrosis (Ormond's Disease)

Idiopathic Retroperitoneal Fibrosis (Ormond's Disease)

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  • Ormond's disease is a diffuse or localized fibroblastic proliferation associated with chronic inflammation.
  • Although benign, this lesion can be locally aggressive, with compression or obstruction of the ureters and vascular structures, including the aorta.
  • Cause is unknown, but frequent association with immune disease and response to steroids suggests being immune-related (hyper-immunoglobulin G4 syndrome: elevated serum IgG4 and plasma cells are IgG4+).
  • Gastrointestinal involvement is unusual, but may occur.
  • Clinical:
    • Usually occurs in adults; rarely in children.
    • Patients present with a history of pain in the lower abdomen, flank, or lower back.
    • One third of cases associated with drugs (esp. methysergide, adrenergic blocking agents and methyldopa).
    • May occur with malignancies (lymphoma, sarcoma or carcinoma), trauma or immune-mediated connective tissue disease.
    • May be a part of multiple fibrosclerosis (systemic idiopathic fibrosis).

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  • Gross: ill-defined fibrous mass usually centered at the 4th and 5th lumbar vertebrae and often encasing one or both ureters (hydronephrosis) and/or vasculature as well (image A).
  • Histology:
    • Fibroblastic proliferation with densely hyalinized collagen.
    • Variable (but generally obvious) chronic inflammatory infiltrate composed of lymphocytes and plasma cells (image B), (image C), & (image D).
    • IgG4 positive plasma cells are present.
    • Germinal center formation may occur with marked inflammation.