Topic Highlight
Copyright ©2008 The WJG Press and Baishideng.
World J Gastroenterol. Jun 7, 2008; 14(21): 3350-3359
Published online Jun 7, 2008. doi: 10.3748/wjg.14.3350
Table 5 Comparison of PSC and AIP-SC
GenderM:F = 2:1Probably some male predominance[818587]
Clinical presentationUsually insidious. Sometimes with obstructive jaundice secondary to cholangiocarcinoma.Mild abdo/Back pain
Sometimes with short history of obstructive jaundice due to CBD stricture
Associated inflammatory bowel diseaseYesNo
Cholangiographic findingsDiffuse changes throughout intra- and extrahepatic bile ducts. Abnormalities in pancreatic duct common.Pancreatic duct strictures or narrowing. Often stricture of distal 1/3 of common bile duct. Intrahepatic duct changes less common.
Blood chemistry dataOften cholestatic but bilirubin usually near normal.May be cholestatic. Bilirubin often high
AutoantibodiesAtypical pANCA plus range of othersAntibodies to carbonic anhydrase II plus range of others[808184]
ImmunoglobulinsIgG4 levels normalIgG4 levels usually elevated[82]
HistologyAbsence of plasma cells positive for IgG4 on immunostainingIgG4 positive plasma cells present in bile ducts and portal tracts[79]
Liver biopsy stagingRange of Ludwig staging including higher stages eg III or IVLudwig staging usually only I or II[86]
TreatmentUrsodeoxycholic acid ± biliary drainage for dominant stricturesSystemic steroid therapy usually leads to complete resolution of symptoms and signs of disease. Occasionally patients relapse and require longer courses of steroids