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Pericholangitis

Background

Historically, the term pericholangitis has referred to the entity of chronic hepatitis and cholestatic biochemical features typically occurring in patients with inflammatory bowel disease (IBD). However, the nomenclature of this disease has come into question because of the recognition that pericholangitis and classic (or large-duct) primary sclerosing cholangitis (PSC) might be parts of a disease spectrum.

In particular, studies comparing patients who have chronic IBD, cholestatic liver biochemistry, and typical cholangiographic evidence of PSC with similar patients who have normal findings on cholangiography reveal similarities in clinical features and liver biopsy findings. Furthermore, some patients with pericholangitis and initially negative findings on cholangiography have been demonstrated on long-term follow-up to develop typical cholangiographic features of PSC.

For this reason, the disease previously termed pericholangitis is now more properly referred to as small-duct PSC. For the purpose of this article, these terms are used interchangeably. Pericholangitis and large-duct PSC should be considered as part of a disease spectrum, best referred to as PSC with small-duct involvement, large-duct involvement, or both.

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