Review
Copyright ©The Author(s) 2016.
World J Hepatol. Feb 18, 2016; 8(5): 265-272
Published online Feb 18, 2016. doi: 10.4254/wjh.v8.i5.265
Table 1 Differential diagnosis for primary sclerosing cholangitis
VascularHepatic artery thrombosis
Portal hypertension bilopathy
Portal cavernoma associated cholangiopathy
Intra-arterial chemotherapy
Sickle cell disease related cholangiopathy
TraumaTrauma post cholecystectomy
Abdominal trauma
InfectionsAIDS related cholangiopathy
Recurrent pyogenic cholangitis
BenignIntraductal stone disease
MalignancyCholangiocarcinoma
AutoimmuneAutoimmune sclerosing cholangitis
IgG4 related sclerosing cholangitis
Systemic vasculitis
OtherRecurrent pancreatitis
Sclerosing cholangitis in critically ill patient
TPN related cholangiopathy
Histocytosis X
Table 2 Primary sclerosing cholangitis compared to immunoglobulin G4 related sclerosing cholangitis (adapted from Joshi 2014)
PSCIAC
Gender (M:F)1.5:17:1
Age of onsetYoung (< 40 yr)Older (> 50 yr)
PresentationCholestatic liver enzymesObstructive jaundice
CholangiogramBeading, band-like strictures, peripheral pruningLong smooth strictures, low CBD strictures
CholangioscopyDilated and tortuous vesselsScarring, pseudo diverticula
Raised serum IgG4 levels< 20%> 70%
Pancreatic involvement< 5%> 80%
Cholangiocarcinoma9%Rare
Association with IBD80%< 10%
Response to steroidsRare (IgG4 + ve PSC)97%
Table 3 Important studies involving ursodeoxycholic acid
Ref.Dose and study designNumber UDCA (number placebo)Follow upParameterOutcome
Chazouillères et al[51]8-16 mg/kg156 moLiver enzymesImproved
UDCA alone
O'Brien et al[52]10 mg/kg1237 moLiver enzymesImproved
UDCA alone
Beuers et al[53]13-15 mg/kg6 (8)12 moLiver enzymesImproved
UDCA vs placeboHistologyImproved
Lindor et al[54]13-15 mg/kg51 (51)2.2 yrLiver enzymesImproved
UDCA v placeboTime to treatment failureNo change
Time to liver transplantNo change
Olsson et al[55]17-23 mg/kg110 (109)5 yrLiver enzymesNo change
UDCA vs placeboTransplant free survivalNo change
Lindor et al[56]28-30 mg/kg76 (74)TerminatedLiver enzymesImproved
UDCA vs placeboPrimary end-pointIncreased
Serious adverse eventsIncreased
Table 4 Mayo risk score1
ParameterWeighting
Age+ 0.03 × absolute value
Bilirubin+ 0.54 × log
Aspartate aminotransferase+ 0.54 × log
Variceal bleeding+ 1.24 × yes/no
Albumin- 0.84 × absolute value