Idiopathic retroperitoneal fibrosis

Idiopathic

retroperitoneal fibrosis • Mistletoe sign - Ganzer Fall bei Radiopaedia

Suhai, F. Mistletoe sign. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-85301 • CC by-nc-sa 3.0 (modified)

Retroperitoneal

fibrosis • Retroperitoneal fibrosis on intravenous pyelogram - Ganzer Fall bei Radiopaedia

Gerstenmaier J, Retroperitoneal fibrosis on intravenous pyelogram. Case study, Radiopaedia.org (Accessed on 15 Aug 2023) https://doi.org/10.53347/rID-24630 • CC by-nc-sa 3.0 (modified)

Use of

rituximab in idiopathic retroperitoneal fibrosis. CT scan of Patient 2 pre-treatment (a) demonstrating a soft tissue mass (arrow) in keeping with RPF partially encasing the aorta, IVC and the right ureter. CT scan performed subsequently in the same patient post treatment (b) demonstrates that the soft tissue representing RPF has almost completely resolved. There is only a small amount of residual tissue (arrow) surrounding the right ureter, which has a stent in place

bmcrheumatol.biomedcentral.com • CC-by-4.0

A case of

idiopathic retroperitoneal fibrosis presenting as gallbladder carcinoma. Characteristics of the abdominal CT scan. Abdominal computed tomography scan showing the gallbladder occupying and involving the hilar bile duct and dilatation of the proximal biliary system (a); posterior peritoneal mass occupying/involving the upper part of the bilateral ureter (b), and the change in the proximal urinary tract (c)

bmcsurg.biomedcentral.com • CC-by-4.0

Radiopaedia • CC-by-nc-sa 3.0 • de

Idiopathic retroperitoneal fibrosis, also known as Ormond disease, is a subtype of retroperitoneal fibrosis where no obvious cause is found. It includes a spectrum of diseases which are characterized 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.

Epidemiology

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

Associations

Reported associations include

Pathology

This inflammatory pathology is thought to arise as an autoimmune response to ceroid which leaks out of the atherosclerotic plaques and causes vasculitis. It is seen in association with other autoimmune processes, suggesting the possibility of an underlying autoimmune pathology.

Radiographic features

Contrast-enhancing fibrosis encompassing the retroperitoneal structures causing ureteric and vascular obstruction and displacement is seen with no other demonstrable alternative conditions or malignancy.

Recently contrast enhancing pericoronary 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) .