Clinical profile and outcome of primary sclerosing cholangitis: A single-centre experience from western India
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Primary sclerosing cholangitis (PSC), a chronic progressive cholestatic liver disease of unknown cause, is uncommon in India. The aim of this study was to define the profile and outcomes of patients with PSC in a tertiary centre from western India.
A retrospective study of the prospectively maintained liver clinic database was searched for cases of PSC between January 2008 and December 2017 with minimum 6 months follow up. All cases were reviewed for clinical profile, inflammatory bowel disease (IBD) co-morbidity and major endpoints like death, cholangiocarcinoma and liver transplantation (LT).
We identified 28 (18 men) patients with PSC (19, 67% large-duct and 9, 33% small-duct) with a median age of 31.5 years (range 7–63 years) with median duration of follow up of 24 months (6–125 months). Six (21.4%) had autoimmune hepatitis (AIH-PSC) overlap. Inflammatory bowel disease was seen in 12 (43%) cases, all were ulcerative colitis (UC). During follow up, seven patients (25%) developed dominant stricture or recurrent cholangitis, 11 (39%) had portal hypertension, 2 (7%) developed cholangiocarcinoma and 5 (17.8%) progressed to hepatic decompensation on follow up. Ten (35%) patients died, 5 from liver-related complications, 2 from cholangiocarcinoma, 1 each from brain hemorrhage and systemic sepsis and 1 due to unknown cause; 3 underwent liver transplantation. Revised Mayo score of patients who survived was lower than those who died (1.03 vs. 1.86, p value 0.03).
PSC commonly presents in young age and rapidly progresses to decompensation. Prevalence of IBD in PSC is lower and the proportion of small-duct PSC is higher than that observed in western populations.
KeywordsCholangiocarcinoma Cirrhosis Dominant stricture Inflammatory bowel disease Liver transplantation Mayo prognostic score Portal hypertension
AS and SB have contributed to the study concept, finalizing the protocol, critical revision of the manuscript for intellectual content, technical or material support and study supervision.
PT, PB, AJ and DG have contributed to the study concept and protocol design, collecting data, analysis of the data and preparing the initial draft of the manuscript.
Compliance with ethical standards
Conflict of interest
PT, PB, AJ, DG, SB, and AS declare that they have no conflict of interest.
The study was performed in amanner to conform with the Helsinki Declaration of 1975, as revised in 2000 and 2008 concerning human and animal rights, and the authors followed the policy concerning informed consent as shown on Springer.com. The study was conducted after obtaining proper ethical clearance from the institutional ethics committee.
The authors are solely responsible for the data and the content of the paper. In no way, the Honorary Editor-in-Chief, Editorial Board Members, or the printer/publishers are responsible for the results/ findings and content of this article.
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