Primary Biliary Cholangitis Scoring System (2013)
Grading (Necroinflammatory Activity)
Score
Criterion
CA (Cholangitis Activity)
0 (no activity)
No cholangitis but mild damage to the bile ducts may be present.
1 (mild activity)
One evident chronic cholangitis in the specimen (i.e., nonspecific chronic cholangitis surrounded by mild to moderate lymphoplasmacytic inflammation ).
2 (moderate activity)
Two or more bile ducts with evident chronic cholangitis (i.e., nonspecific chronic cholangitis surrounded by mild to moderate lymphoplasmacytic inflammation ).
3 (marked activity)
One or more foci of chronic nonsuppurative destructive cholangitis in the specimen (i.e., marked epithelial injury with adjacent marked lymphoplasmacytic inflammation or granuloma; florid duct lesion).
HA (Hepatitis Activity)
0 (no activity)
No interface hepatitis and no or minimal lobular hepatitis
1 (mild activity))
Interface hepatitis affecting 10 continuous hepatocytes at a limiting plate in 1 portal tract or fibrous septa and mile to moderate lobular hepatitis.
2 (moderate activity)
Interface hepatitis affecting 10 continuous hepatocytes at a limiting plate in 2 or more portal tracts or fibrous septa and mile to moderate lobular hepatitis.
3 (marked activity)
Interface hepatitis affecting 20 continuous hepatocytes at limiting plates in moree than half of the portal tracts and moderate lobular hepatitis or bridging/zonal necrosis.
Staging (Chronicity Score)
Score
Criterion
Fibrosis
0
No fibrosis or fibrosis limited to portal tracts.
1
Portal fibrosis with periportal fibrosis or incomplete septal fibrosis.
2
Bridging fibrosis with variable lobular disarray.
3
Cirrhosis with regenerative nodules and extensive fibrosis.
Bile Duct Loss
0
No bile duct loss.
1
Bile duct loss in less than one-third of portal tracts.
2
Bile duct loss in one-third to two-thirds of portal tracts.
3
Bile duct loss in more than two-thirds of portal tracts.
Deposition of Orcein-Positive Granules
0
No deposition of granules.
1
Deposition of granules in a couple of Zone 1 hepatocytes in less than one-third of portal tracts.
2
Deposition of granules in a variable number of Zone 1 hepatocytes in one-third to two-thirds of portal tracts.
3
Deposition of granules in most Zone 1 hepatocytes in more than two-thirds of portal tracts.
Reference: Kakuda Y et al. Evaluation of a new histologic staging and grading system for primary biliary cirrhosis in comparison with classical systems. Hum Pathol 2013; 44:1107-1117. Last Modified: Sun May 10 2020 5:55 PM EDT
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