Primary Biliary Cholangitis

 > 90% of patients are female


image Most common in individuals of North European descent


• Insidious onset with pruritus, fatigue, jaundice
image Often other associated autoimmune disorders

• AMA(+)
image Minority of cases are AMA(-)

• Elevation of GGT, alkaline phosphatase
image Out of proportion to transaminases

• Ursodeoxycholic acid is treatment of choice
image Not cure but delays progression in some patients




Microscopic




• Florid duct lesion with lymphocytic cholangitis and bile duct injury
image Granulomatous inflammation variably present

• Biliary epithelial disarray with irregularly sized and pseudostratified nuclei and vacuolated, swollen cytoplasm

• Bile ductular reaction

• Cholate stasis
image Copper stain highlights accumulated copper in hepatocytes

• Initially portal-based fibrosis, eventually forms portal-portal bridges
image Cirrhosis is biliary type with irregular nodules (so-called jigsaw puzzle pattern)

• 4 histologic stages ranging from portal involvement to cirrhosis

image
Gross Specimen
This photograph of the cut surface of a liver explanted for primary biliary cholangitis (PBC) emphasizes the green discoloration of the cirrhotic nodules, indicative of chronic cholestasis.


image
Florid Duct Lesion
This example of a florid duct lesion shows lymphocytic cholangitis in the interlobular bile duct image along with a nodular portal lymphoplasmacytic infiltrate and a granulomatous inflammation.

image
Portal Tract Changes
This biopsy from a patient with PBC shows nodular lymphoplasmacytic inflammation and lymphocytic cholangitis, even at low power. Fibrous expansion of the portal tract is also present. Similar histologic changes are also characteristic of AMA PBC (autoimmune cholangitis).

image
Copper Stain
The copper stain highlights changes of chronic cholestasis consisting of periportal orange-red granules image in the hepatocytes in PBC.


TERMINOLOGY


Abbreviations




• Primary biliary cirrhosis/cholangitis (PBC)
image Cholangitis is recently preferred term


Definitions




• Chronic cholestatic disease in which intrahepatic bile ducts are progressively destroyed by nonsuppurative inflammation


ETIOLOGY/PATHOGENESIS


Unknown




• Probable autoimmune etiology


CLINICAL ISSUES


Epidemiology




• Incidence
image Most common in individuals of North European descent

– Typically 40-60 years of age

– > 90% of patients are female


Presentation




• Insidious onset with pruritus (most common), fatigue, jaundice, associated autoimmune disorders


Laboratory Tests




• AMA(+)

• Elevation of GGT &/or alkaline phosphatase out of proportion to transaminases, which are typically mildly elevated or normal

• Elevated bilirubin, usually late in disease

• Elevated IgM

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Primary Biliary Cholangitis
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