BCM
Gastroenterology Grand Rounds - Take Home Lessons
The main lessons to take home concerning this case
are:
1. PSC is a rare form of progressive biliary inflammation
and fibrosis of unknown etiology associated with IBD and characterized
by intra- and/or extrahepatic biliary duct destruction that ultimately
leads to cirrhosis and end-stage liver failure.
2. Review of the limited reported cases in the literature
indicate that in patients with PSC, pregnancy does not seem to have a negative
effect on the disease process itself or carry a worse outcome for the fetus.
3. Diagnostic and therapeutic ERCP appear reasonably
safe and effective in pregnancy; use of appropriate shielding, limiting
fluoroscopy time, and use of image intensifier can limit radiation exposure
within the established guidelines.
4. Ursodeoxycholic acid may improve liver histology and
liver function tests; preliminary data suggest an improved survival benefit
at higher dosing.