How is primary biliary cholangitis (PBC) staged?

Updated: Nov 08, 2017
  • Author: Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP, AGAF; Chief Editor: BS Anand, MD  more...
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Answer

Various staging systems have been developed, but the most prominent are those proposed by Ludwig et al and Scheuer, as follows [9] :

  • Stage 1 (portal stage of Ludwig): Portal inflammation, bile duct abnormalities, or both are present.

  • Stage 2 (periportal stage): Periportal fibrosis is present, with or without periportal inflammation or prominent enlargement of the portal tracts with seemingly intact, newly formed limiting plates. See the image below.

    This histologic picture is compatible with stage 2 This histologic picture is compatible with stage 2 primary biliary cholangitis.
  • Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa, or both are present.

  • Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present.


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