Small-duct PSC and its prognosis

What is known

• Small-duct PSC is defined by biochemical chronic cholestasis and liver biopsy findings consistent with PSC but a normal cholangiogram. ^1–3
• Studies published in 2002 and a later combined cohort with prolonged follow-up suggested small-duct PSC has a better prognosis than large-duct PSC. ²
• Cholangiocarcinoma has not been described in small-duct PSC unless it first progresses to large-duct PSC. ^1–3
• Small-duct PSC appears to be a separate entity that rarely progresses to large-duct disease. ^1,3

What is unknown / caveats

• Favorable-prognosis studies were small with short follow-up
• Some patients still develop liver-related mortality, need transplantation, or have recurrence after transplant
• Despite the overall favorable outlook, some small-duct PSC patients progress to large-duct disease, and the natural history is not fully established.

References

1. Nikolaidis NL, Giouleme OI, Tziomalos KA, Patsiaoura K, Kazantzidou E, Voutsas AD, Vassiliadis T, Eugenidis NP. Small-duct primary sclerosing cholangitis. A single-center seven-year experience. Dig Dis Sci. 2005. PMID: 15745094.

2. Björnsson E. Small-duct primary sclerosing cholangitis. Curr Gastroenterol Rep. 2009. PMID: 19166657.

3. Björnsson E, Chapman RW. Sclerosing cholangitis. Curr Opin Gastroenterol. 2003. PMID: 15703568.

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