Idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis, also known as Ormond disease or or occasionally Albarran-Ormond syndrome ⁶, is a subtype of retroperitoneal fibrosis where no cause is found. It includes a spectrum of diseases that are characterised by fibroinflammatory tissue encasing the abdominal aorta and the iliac arteries. This process may extend into the retroperitoneum, often enveloping the adjacent structures, e.g. ureters ureters.

Epidemiology

Retroperitoneal fibrosis in general is an uncommon condition with an estimated incidence of about 1.38 in 100,000 ¹. Of these, ~70% are idiopathic.

Associations

It is seen in association with other autoimmune processes, suggesting the possibility of an underlying autoimmune pathology.

Reported associations include:

• primary biliary cholangitis

• fibrosing mediastinitis

• glomerulonephritis

• panhypopituitarism

• rheumatoid arthritis

• ankylosing spondylitis

• polyarteritis nodosa

• systemic lupus erythematosus (SLE)

• Hashimoto thyroiditis

• IgG4-related disease ⁷

Pathology

This inflammatory pathology is thought to arise as an autoimmune response to ceroid which leaks out of the atherosclerotic plaques and causes vasculitis.

Radiographic features

Appears as a contrast-enhancing fibrosis encompassing the retroperitoneal structures, typically causing ureteric and vascular obstruction and displacement with no other demonstrable alternative conditions or malignancy.

Recently, contrastContrast-enhancing pericoronary artery tissue proliferations (mistletoe sign) have also been reported in some patients.

Treatment and prognosis

Complications such as acute renal failure secondary to periureteral involvement require prompt intervention. Treatment usually involves corticosteroids with or without other immunomodulating medications or tamoxifen. In the presence of periureteral or perivascular involvement, surgical intervention may be necessary.

History and etymology

The disorder was first described in 1905 by the French urologist Joaquin Albarran (1860-1912) and later first described in the English literature in 1948 by the American urologist John Kelso Ormond (1886-1978) ^3-6.