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.
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).
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).
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.