Controversies in the management of primary sclerosing cholangitis

Table 2 Primary sclerosing cholangitis compared to immunoglobulin G4 related sclerosing cholangitis (adapted from Joshi 2014)

	PSC	IAC
Gender (M:F)	1.5:1	7:1
Age of onset	Young (< 40 yr)	Older (> 50 yr)
Presentation	Cholestatic liver enzymes	Obstructive jaundice
Cholangiogram	Beading, band-like strictures, peripheral pruning	Long smooth strictures, low CBD strictures
Cholangioscopy	Dilated and tortuous vessels	Scarring, pseudo diverticula
Raised serum IgG4 levels	< 20%	> 70%
Pancreatic involvement	< 5%	> 80%
Cholangiocarcinoma	9%	Rare
Association with IBD	80%	< 10%
Response to steroids	Rare (IgG4 + ve PSC)	97%

M: Male; F: Female; PSC: Primary sclerosing cholangitis; IgG4: Immunoglobulin G4; IBD: Inflammatory bowel disease.