[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [General pathology](https://durgical.com/en/general-pathology/) › [Liver and gallbladder](https://durgical.com/en/general-pathology/areas/liver/) › Primary biliary cholangitis: the florid duct lesion and AMA

# Primary biliary cholangitis: the florid duct lesion and AMA

- Lesion

In primary biliary cholangitis the immune system destroys the smallest bile ducts, slowly, mostly in middle-aged women.

Updated Nov 12, 2026

## Key points

- Women much more than men; middle age.
- Antimitochondrial antibodies (anti-PDC-E2) in most patients.
- Florid duct lesion: lymphocytes and often a granuloma destroying an interlobular bile duct.
- Labs: high alkaline phosphatase and GGT, high cholesterol, high IgM.
- Symptoms: fatigue, pruritus; later jaundice, xanthomas, osteoporosis, fat-soluble vitamin malabsorption.
- Associations: Sjögren disease, systemic sclerosis, thyroiditis, celiac disease.

## What it is

Primary biliary cholangitis is an autoimmune disease in which T cells destroy small intrahepatic (interlobular) bile ducts. It mainly affects middle-aged women, and almost all patients have antimitochondrial antibodies against the E2 subunit of the pyruvate dehydrogenase complex. Duct loss causes chronic cholestasis that can progress to cirrhosis over years. It is often found by a raised alkaline phosphatase before symptoms.

## Structure

- Early: dense portal lymphoplasmacytic infiltrate around a damaged interlobular duct, often with an epithelioid granuloma.
- Later: duct loss (ductopenia), ductular reaction, copper-associated protein in periportal hepatocytes.
- End stage: green, finely nodular cirrhotic liver.

## Step by step

- Loss of tolerance to a mitochondrial antigen (PDC-E2) expressed by biliary epithelial cells.
- Autoreactive T cells and antibodies target small interlobular ducts.
- Ducts are destroyed; granulomas may form around them (florid duct lesion).
- Bile acids are retained, injuring periportal hepatocytes (cholate stasis).
- Ductular reaction and periportal fibrosis spread.
- Over years septa link portal tracts: biliary cirrhosis.

## Markers

- Antimitochondrial antibody (anti-PDC-E2)
- Keratin 7 (shows ducts and ductules)
- Orcein (copper-associated protein)

## Memory aid

PBC: Pretty Bad for the smallest Channels, in middle-aged women with Mitochondrial antibodies.

## Test yourself

Think of the answer, then tap the question to see it.

## Which autoantibody is characteristic of primary biliary cholangitis?

Antimitochondrial antibody against the E2 subunit of the pyruvate dehydrogenase complex.

## What is the florid duct lesion?

Destruction of an interlobular bile duct by dense lymphocytic, often granulomatous, inflammation, the hallmark of PBC.

## Which ducts are destroyed in PBC: large or small?

Small intrahepatic interlobular bile ducts; large ducts are spared.

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## Elsewhere in DurgiPedia

- [Immune tolerance and how autoimmunity begins](https://durgical.com/en/immunology/immune-tolerance-and-how-autoimmunity-begins/)
- [Gallbladder and bile ducts](https://durgical.com/en/histology/gallbladder-and-bile-ducts/)

## Connections

## Related 4

- [Primary sclerosing cholangitis](https://durgical.com/en/general-pathology/primary-sclerosing-cholangitis/)
- [Vanishing bile duct syndrome](https://durgical.com/en/general-pathology/vanishing-bile-duct-syndrome/)
- [Autoimmune hepatitis: plasma cells, interface injury and types 1 and 2](https://durgical.com/en/general-pathology/autoimmune-hepatitis-plasma-cells-interface-injury-and-types-1-and-2/)
- [Granulomatous inflammation: caseating vs non-caseating, the causes to know](https://durgical.com/en/general-pathology/granulomatous-inflammation-caseating-vs-non-caseating-the-causes-to-know/)

## Related conditions 6

- [Primary biliary cholangitis](https://durgical.com/en/conditions/primary-biliary-cholangitis/)
- [Sjögren disease](https://durgical.com/en/conditions/sjogren-disease/)
- [Systemic sclerosis (scleroderma, including CREST)](https://durgical.com/en/conditions/systemic-sclerosis/)
- [Thyroiditis (subacute, painless and postpartum)](https://durgical.com/en/conditions/thyroiditis/)
- [Coeliac disease](https://durgical.com/en/conditions/coeliac-disease/)
- [Cirrhosis](https://durgical.com/en/conditions/cirrhosis/)

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: [Primary biliary cholangitis: MedlinePlus Medical Encyclopedia](https://medlineplus.gov/ency/article/000282.htm) (MedlinePlus, US National Library of Medicine, 2026).

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Representative model, for educational purposes only.

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License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/general-pathology/primary-biliary-cholangitis-the-florid-duct-lesion-and-ama/