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# Autoimmune hepatitis: plasma cells, interface injury and types 1 and 2

- Lesion

In autoimmune hepatitis the immune system attacks the person's own liver cells; untreated it often leads to cirrhosis.

Updated Nov 12, 2026

## Key points

- Type 1 (most common, adults): ANA and anti-smooth muscle (anti-actin) antibodies; sometimes anti-SLA/LP.
- Type 2 (mostly children): anti-LKM-1 (against CYP2D6) and anti-liver cytosol 1 antibodies.
- Histology: plasma-cell-rich interface hepatitis, rosettes of hepatocytes, emperipolesis, often confluent necrosis.
- Raised serum IgG supports the diagnosis.
- Associated with other autoimmune diseases (thyroiditis, type 1 diabetes, celiac disease) and HLA-DR3/DR4.
- Some drugs (minocycline, nitrofurantoin, methyldopa) cause an autoimmune-like hepatitis that resolves when stopped.

## What it is

Autoimmune hepatitis is a chronic hepatitis caused by a T-cell attack on hepatocytes, with autoantibodies and raised IgG. It is more common in women, can present at any age, and ranges from abnormal tests to acute liver failure or cirrhosis at diagnosis. The diagnosis combines autoantibodies, high IgG, a compatible biopsy and the exclusion of viral and drug causes. It responds to immunosuppression, so recognizing it matters.

## Structure

- Dense portal and interface infiltrate of lymphocytes with many plasma cells in clusters.
- Hepatocyte rosettes: small circles of regenerating hepatocytes around a dilated canaliculus.
- Emperipolesis: lymphocytes or plasma cells inside hepatocyte cytoplasm.
- Confluent perivenular, bridging or panacinar necrosis in severe flares; fibrosis or even burnt-out cirrhosis at first biopsy.

## Step by step

- A genetically susceptible person (certain HLA alleles) loses tolerance to liver antigens.
- A trigger is suspected (virus, drug), but the target antigen is usually unknown.
- CD4 helper T cells drive B cells to make autoantibodies and plasma cells gather in portal tracts.
- CD8 cytotoxic T cells kill hepatocytes at the interface.
- Repeated injury causes fibrosis and, without treatment, cirrhosis.

## Markers

- ANA
- Anti-smooth muscle (F-actin)
- Anti-LKM-1
- Anti-SLA/LP
- Serum IgG

## Clinical notes

- Presentations: asymptomatic raised aminotransferases, acute hepatitis, acute liver failure, or established cirrhosis.
- Overlap features with primary biliary cholangitis or primary sclerosing cholangitis (the latter mostly in children) occur.

## Memory aid

Autoimmune hepatitis is a plasma-cell party at the portal border: invitation by ANA and smooth-muscle antibodies, a bill paid in IgG.

## Test yourself

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

## Which antibodies characterize type 1 autoimmune hepatitis?

Antinuclear antibodies and anti-smooth muscle (anti-actin) antibodies.

## Which antibody defines type 2 autoimmune hepatitis and what is its target?

Anti-liver kidney microsome type 1, directed against cytochrome P450 2D6.

## What histologic feature most supports autoimmune hepatitis over viral hepatitis?

Clusters of plasma cells in a florid interface hepatitis, with rosettes and emperipolesis.

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## Elsewhere in DurgiPedia

- [Immune tolerance and how autoimmunity begins](https://durgical.com/en/immunology/immune-tolerance-and-how-autoimmunity-begins/)
- [HLA associations with disease](https://durgical.com/en/immunology/hla-associations-with-disease/)

## Connections

## Related 5

- [Viral hepatitis on biopsy: acute and chronic](https://durgical.com/en/general-pathology/viral-hepatitis-on-biopsy-acute-and-chronic/)
- [Primary biliary cholangitis: the florid duct lesion and AMA](https://durgical.com/en/general-pathology/primary-biliary-cholangitis-the-florid-duct-lesion-and-ama/)
- [Primary sclerosing cholangitis](https://durgical.com/en/general-pathology/primary-sclerosing-cholangitis/)
- [Drug- and toxin-induced liver injury](https://durgical.com/en/general-pathology/drug-and-toxin-induced-liver-injury/)
- [Chronic inflammation: causes, cells and features](https://durgical.com/en/general-pathology/chronic-inflammation-causes-cells-and-features/)

## Related conditions 6

- [Autoimmune hepatitis](https://durgical.com/en/conditions/autoimmune-hepatitis/)
- [Acute liver failure](https://durgical.com/en/conditions/acute-liver-failure/)
- [Cirrhosis](https://durgical.com/en/conditions/cirrhosis/)
- [Type 1 diabetes mellitus](https://durgical.com/en/conditions/type-1-diabetes-mellitus/)
- [Coeliac disease](https://durgical.com/en/conditions/coeliac-disease/)
- [Thyroiditis (subacute, painless and postpartum)](https://durgical.com/en/conditions/thyroiditis/)

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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: [Autoimmune hepatitis: MedlinePlus Medical Encyclopedia](https://medlineplus.gov/ency/article/000245.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/autoimmune-hepatitis-plasma-cells-interface-injury-and-types-1-and-2/