Controversies in the management of primary sclerosing cholangitis

Table 1 Differential diagnosis for primary sclerosing cholangitis

Vascular	Hepatic artery thrombosis
	Portal hypertension bilopathy
	Portal cavernoma associated cholangiopathy
	Intra-arterial chemotherapy
	Sickle cell disease related cholangiopathy
Trauma	Trauma post cholecystectomy
	Abdominal trauma
Infections	AIDS related cholangiopathy
	Recurrent pyogenic cholangitis
Benign	Intraductal stone disease
Malignancy	Cholangiocarcinoma
Autoimmune	Autoimmune sclerosing cholangitis
	IgG4 related sclerosing cholangitis
	Systemic vasculitis
Other	Recurrent pancreatitis
	Sclerosing cholangitis in critically ill patient
	TPN related cholangiopathy
	Histocytosis X

IgG4: Immunoglobulin G4; TPN: Total parenteral nutrition; AIDS: Acquired immune deficiency syndrome.

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.

Table 3 Important studies involving ursodeoxycholic acid

Ref.	Dose and study design	Number UDCA (number placebo)	Follow up	Parameter	Outcome
Chazouillères et al[51]	8-16 mg/kg	15	6 mo	Liver enzymes	Improved
	UDCA alone
O'Brien et al[52]	10 mg/kg	12	37 mo	Liver enzymes	Improved
	UDCA alone
Beuers et al[53]	13-15 mg/kg	6 (8)	12 mo	Liver enzymes	Improved
	UDCA vs placebo			Histology	Improved
Lindor et al[54]	13-15 mg/kg	51 (51)	2.2 yr	Liver enzymes	Improved
	UDCA v placebo			Time to treatment failure	No change
				Time to liver transplant	No change
Olsson et al[55]	17-23 mg/kg	110 (109)	5 yr	Liver enzymes	No change
	UDCA vs placebo			Transplant free survival	No change
Lindor et al[56]	28-30 mg/kg	76 (74)	Terminated	Liver enzymes	Improved
	UDCA vs placebo			Primary end-point	Increased
				Serious adverse events	Increased

UDCA: Ursodeoxycholic acid.

Table 4 Mayo risk score¹

Parameter	Weighting
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

¹The link is: http://www.mayoclinic.org/medical-professionals/model-end-stage-liver-disease/revised-natural-history-model-for-primary-sclerosing-cholangitis.