Urology>>>>>Renal Pathophysiology
Question 7#

Regarding retroperitoneal fibrosis (RPF), which of the following is FALSE?

A. One possible pathogenesis could be the development of vasculitis in the adventitial vessels of the aorta and periaortic small vessels
B. Inflammatory bowel disease (IBD) is reported to be a cause of RPF
C. The classic radiologic findings include medial deviation of extrinsically compressed ureters with hydronephrosis
D. CT typically reveals a well demarcated retroperitoneal mass, isodense to muscle on unenhanced studies
E. . Tamoxifen, a steroidal anti-oestrogen, has also been used for primary treatment at a dose of 20 mg per day with a reported response rate of 80%

Correct Answer is E

Comment:

Answer E

The development of vasculitis in the adventitial vessels leading to the release of antigens from atheromatous plaque such as ceroid, a complex lipoprotein, which induces an autoimmune antigenic response. The reported inflammatory conditions associated with RPF are IBD, ascending lymphangitis, asbestosis, amyloidosis and sarcoidosis. The classic radiologic findings of medial deviation of extrinsically compressed ureters can be demonstrated in up to 18% of normal subjects. CT reveals a well-defined mass, isodense with muscle in plain films. Tamoxifen is a nonsteroidal anti-oestrogen and is suggested to alter TGF-β, thereby limiting fibrosis. E. F. van Bommel reported a response rate of 74% in patients treated with Tamoxifen 20 mg OD for median duration of 8.5 months.