Small-duct primary sclerosing cholangitis

A long-term follow-up study

Paul Angulo, Yaakov Maor-Kendler, Keith Lindor

Research output: Contribution to journalArticle

154 Citations (Scopus)

Abstract

Some patients with inflammatory bowel disease (IBD) have chronic cholestasis and hepatic histology compatible with primary sclerosing cholangitis (PSC) but normal findings on cholangiography. These patients with small-duct PSC have remained largely unstudied. Our aim was to determine the prevalence and long-term outcomes of patients with small-duct PSC. Eighteen patients with small-duct PSC (7 female and 11 male patients; mean age, 39.9 ± 15.3 years [range, 13-68 years]) seen over a 4-year period were matched blindly by age and sex to 36 patients with classic PSC and followed up for 32.5 years. Small-duct PSC represented 5.8% of patients (18 of 309) with sclerosing cholangitis. Subsequent endoscopic retrograde cholangiography (ERC) performed in 5 patients with small-duct PSC showed progression to typical PSC in 3 patients at 4, 5.5, and 21 years of follow-up. None of the patients with small-duct PSC but 4 of the patients with classic PSC developed hepatobiliary malignancy. There were 3 deaths (17%) or liver transplantations in patients with small-duct PSC (2 after progressing to classic PSC) and 15 (42%) in the classic PSC group. Survival free of liver transplantation was significantly greater in the small-duct than in the classic PSC group (P = .04). Compared with the general U.S. population, survival in patients with small-duct PSC was similar (P = .4) but significantly lower in patients with classic PSC (P < .001). In conclusion, small-duct PSC may represent an earlier stage of PSC associated with a significantly better long-term prognosis. Some patients, however, progress to classic PSC and/or end-stage liver disease with the consequent necessity of liver transplantation.

Original languageEnglish (US)
Pages (from-to)1494-1500
Number of pages7
JournalHepatology
Volume35
Issue number6
DOIs
StatePublished - Jun 2002
Externally publishedYes

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Sclerosing Cholangitis
Liver Transplantation
Cholangiography

ASJC Scopus subject areas

  • Hepatology

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Small-duct primary sclerosing cholangitis : A long-term follow-up study. / Angulo, Paul; Maor-Kendler, Yaakov; Lindor, Keith.

In: Hepatology, Vol. 35, No. 6, 06.2002, p. 1494-1500.

Research output: Contribution to journalArticle

Angulo, Paul ; Maor-Kendler, Yaakov ; Lindor, Keith. / Small-duct primary sclerosing cholangitis : A long-term follow-up study. In: Hepatology. 2002 ; Vol. 35, No. 6. pp. 1494-1500.
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abstract = "Some patients with inflammatory bowel disease (IBD) have chronic cholestasis and hepatic histology compatible with primary sclerosing cholangitis (PSC) but normal findings on cholangiography. These patients with small-duct PSC have remained largely unstudied. Our aim was to determine the prevalence and long-term outcomes of patients with small-duct PSC. Eighteen patients with small-duct PSC (7 female and 11 male patients; mean age, 39.9 ± 15.3 years [range, 13-68 years]) seen over a 4-year period were matched blindly by age and sex to 36 patients with classic PSC and followed up for 32.5 years. Small-duct PSC represented 5.8{\%} of patients (18 of 309) with sclerosing cholangitis. Subsequent endoscopic retrograde cholangiography (ERC) performed in 5 patients with small-duct PSC showed progression to typical PSC in 3 patients at 4, 5.5, and 21 years of follow-up. None of the patients with small-duct PSC but 4 of the patients with classic PSC developed hepatobiliary malignancy. There were 3 deaths (17{\%}) or liver transplantations in patients with small-duct PSC (2 after progressing to classic PSC) and 15 (42{\%}) in the classic PSC group. Survival free of liver transplantation was significantly greater in the small-duct than in the classic PSC group (P = .04). Compared with the general U.S. population, survival in patients with small-duct PSC was similar (P = .4) but significantly lower in patients with classic PSC (P < .001). In conclusion, small-duct PSC may represent an earlier stage of PSC associated with a significantly better long-term prognosis. Some patients, however, progress to classic PSC and/or end-stage liver disease with the consequent necessity of liver transplantation.",
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