Retroperitoneal Fibrosis

Excerpt

Retroperitoneal fibrosis is a relatively rare immune-mediated condition characterized by a chronic, progressive periaortic inflammatory and fibrotic process in the retroperitoneum, which can lead to the compression of retroperitoneal structures such as the ureters, aorta, and vena cava. This fibrotic process can often result in the encasement and extrinsic compression of the ureters and other retroperitoneal structures, including the aorta and inferior vena cava. The condition may be idiopathic, drug-related, or secondary to immune-mediated disease.

Retroperitoneal fibrosis may be asymptomatic but typically presents with dull pain or discomfort in the abdomen, back, flank, or inguinal area. Additional common nonspecific signs include hypertension and some degree of azotemia. Diagnosis is often made incidentally during studies conducted for unrelated reasons, and a tissue biopsy may be necessary in some cases. Treatment primarily involves medical therapy, such as steroids, though some cases may require surgical management.

Retroperitoneal fibrosis was originally called Ormond disease, named after John Ormond, an American urologist who described two cases in 1948. However, the first reported case was documented by French urologist Joaquin Albarran in 1905. Older terms for retroperitoneal fibrosis, which are no longer used, include chronic periureteritis, fibrous retroperitonitis, Ormond disease, periureteritis fibrosis, and periureteritis plastica.