RT Book, Section
A1 Greenberger, Norton J.
A1 Saltzman, John R.
A2 Greenberger, Norton J.
A2 Blumberg, Richard S.
A2 Burakoff, Robert
SR Print(0)
ID 55962059
T1 Chapter 52. Primary Sclerosing Cholangitis
T2 CURRENT Diagnosis & Treatment: Gastroenterology, Hepatology, & Endoscopy, 2e
YR 2012
FD 2012
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-176848-1
LK accessmedicine.mhmedical.com/content.aspx?aid=55962059
RD 2024/04/24
AB Progressive  inflammatory,  sclerosing,  and  obliterative  disease  of  the  extrahepatic  or  intrahepatic  bile  ducts,  or  both.Disproportionate  elevation  of  serum  alkaline  phosphatase  (4–10  times  normal)  is  seen  in  almost  all  patients.MRCP  or  ERCP  shows  multifocal  stricturing  of  the  intrahepatic  and  extrahepatic  bile  ducts.Small  duct  disease  is  diagnosed  by  liver  biopsy.Liver  biopsy  findings  that  are  highly  specific  include  fibrous  obliteration  of  small  bile  ducts  with  concentric  replacement  by  connective  tissue  in  an  "onion  skin"  pattern.Patients  have  an  8–15%  lifetime  risk  of  developing  cholangiocarcinoma,  and  it  is  often  difficult  to  distinguish  a  dominant  stricture  from  a  cholangiocarcinoma.