Primary sclerosing cholangitis

Diagnostic criteria for primary sclerosing cholangitis: summary of a workshop[1]LaRusso NF, Shneider BL, Black D, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology. 2006 Sep;44(3):746-64. http://www.ncbi.nlm.nih.gov/pubmed/16941705?tool=bestpractice.com ​​

The major criteria for the diagnosis are:

• Cholangiography with multifocal stricturing of the intra- and/or extrahepatic bile ducts

• Biochemical evidence of cholestasis

• Exclusion of secondary causes.

Histologic staging[44]Ludwig J, Barham SS, LaRusso NF, et al. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology. 1981 Nov-Dec;1(6):632-40. http://www.ncbi.nlm.nih.gov/pubmed/7308996?tool=bestpractice.com

• Stage 1: bile duct injury and portal inflammation with minimal fibrosis.

• Stage 2: expansion of portal tracts, periportal fibrosis, and inflammation.

• Stage 3: fibrous septa, bridging fibrosis, more prominent ductopenia.

• Stage 4: cirrhosis.