Imaging Pearls ❯ GU Misc ❯ Retroperitoneal Fibrosis
|
-- OR -- |
|
- Retroperitoneal Fibrosis: Associated Causes
(1) infections such as tuberculosis, histoplasmosis, and actinomycosis;
(2) drugs including methysergide, pergolide, bromocriptine, ergotomines, methyldopa, hydralazine and beta blockers;
(3) external beam radiation that may be used to treat various forms of cancer;
(4) recent abdominal or pelvic surgery; or recent abdominal/pelvic trauma. - “Retroperitoneal fibrosis (RPF) is a rare disease that is marked by systemic inflammation and the development of a periaortic fibroinflammatory mass. The fibroinflammatory infiltration can encase the abdominal aorta, ureters, and other abdominal organs. The clinical presentation often includes constitutional symptoms, abdominal pain, and signs of renal insufficiency or renal failure related to ureteral obstruction. Less frequently, RPF may present with vascular complications, such as venous thrombosis or claudication.”
Retroperitoneal fibrosis. Tzou M, Gazeley DJ, Mason PJ Vasc Med. 2014 Oct;19(5):407-14. - “The idiopathic form of RPF is most common but secondary forms have been described and are associated with malignancy and a variety of different medications. The pathophysiology is uncertain, but RPF has been linked with periaortitis and IgG4-related disease. Treatment centers on the relief of symptoms and complications associated with mass effects. Corticosteroids and other immunosuppressant therapies can improve constitutional symptoms, reduce infiltrate mass, and achieve disease remission, but a chronic relapsing course is not uncommon.” Retroperitoneal fibrosis. Tzou M, Gazeley DJ, Mason PJ Vasc Med. 2014 Oct;19(5):407-14.
- “Retroperitoneal fibrosis (RPF) is a condition characterized by the presence of inflammation and fibrosis in the retroperitoneal space, for which no standard diagnostic criteria exist. Historically, treatment has focused on relieving the obstruction with percutaneous or cystoscopic assisted placement of ureteral stents followed by more definitive resolution of ureteric obstruction with open or laparoscopic ureterolysis. However, over the past several years management has shifted from primarily a surgical approach to an immunosuppressive-based therapy aimed at modulation of the immune system.”
Retroperitoneal fibrosis. Scheel PJ Jr, Feeley N Rheum Dis Clin North Am. 2013 May;39(2):365-81.