Pratik Tibdewal1, Pratin Bhatt1, Abhinav Jain1, Deepak Gupta1, Shobna Bhatia1, Akash Shukla2. 1. Department of Gastroenterology, Seth GS Medical College, and KEM Hospital, Mumbai 400 012, India. 2. Department of Gastroenterology, Seth GS Medical College, and KEM Hospital, Mumbai 400 012, India. drakashshukla@yahoo.com.
Abstract
INTRODUCTION: 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. METHODS: 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). RESULTS: 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). CONCLUSION: 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.
INTRODUCTION: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. METHODS: 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). RESULTS: 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). CONCLUSION: 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.
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