QuestionSBA
QuestionChoice
This is associated with type I AIH. Anti-smooth muscle antibodies are also associated with type I AIH
31923
a
Anti-nuclear antibodies (ANA)
QuestionChoice
This antibody is associated with rheumatoid arthritis
31926
d
Anti-cyclic citrullinated peptide antibody
QuestionChoice
This is associated with myositis (e.g. dermatomyositis)
31927
e
Anti-mi-2
QuestionChoice
This is associated with type II AIH which only affects children
31924
b
Anti-liver kidney microsomal type 1 (anti-LKM-1)
QuestionChoice
This is associated with type III AIH
31925
c
Soluble liver-kidney antigen
QuestionComment
ANA Loves Smooth Liver" to remember that Anti-Nuclear Antibodies (ANA) and Anti-Smooth Muscle Antibodies are associated with Type I Autoimmune Hepatitis (AIH).
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# Summary

Autoimmune hepatitis is a condition typically seen in young and middle-aged women, characterised by inflammation of the liver caused by an autoimmune response. Key signs and symptoms include jaundice, fatigue, hepatomegaly, and abdominal pain. Key investigations include liver function tests which often show a hepatic pattern of disease and hypergammaglobulinemia. The main types of autoimmune hepatitis are Type I, II, and III, identified by different antibody profiles. Management strategies often depend on the severity of symptoms and disease, and typically include steroid induction therapy followed by maintenance therapy with azathioprine.

# Definition

Autoimmune hepatitis is a chronic, inflammatory disease of the liver that occurs when the body's immune system attacks liver cells leading to inflammation and damage. It is classified into three main types based on the presence of specific antibodies.

# Epidemiology

Autoimmune hepatitis is most frequently observed in young and middle-aged women. There is a notable association with other autoimmune disorders, including: pernicious anaemia, ulcerative colitis, Hashimoto’s/Grave’s, autoimmune haemolytic anaemia, primary sclerosing cholangitis.

# Aetiology

Autoimmune hepatitis occurs due to an abnormal autoimmune reaction against hepatocyte surface antigen, with HLA-B8 and DR3 most frequently implicated. Specifically, there are 3 different types of autoimmune hepatitis:

Type 1) ANA positive, with anti-smooth muscle antibodies

Type 2) anti-liver/kidney mitochondrial type 1 antibodies (LKM1) – can be remembered as ‘Little Kids’ – children predominantly get Type 2

Type 3) Antibodies directed against soluble liver-kidney Antigen 

# Signs and Symptoms

- Can present as an acute hepatitis - fever, jaundice, malaise, abdominal pain urticarial rash, polyarthritis, pulmonary infilitration, glomerulonephritis
- It can also present as chronic liver disease - ascites, jaundice, leuconychia, spider naevi  

Other signs and symptoms include:

- Fatigue
- Anorexia
- Hepatomegaly
- Splenomegaly


# Differential Diagnosis

The main differentials for autoimmune hepatitis are other causes of:

- Acute hepatitis e.g. viral A/E/B, drugs (paracetamol poisoning), ischaemia
- Chronic liver disease e.g. alcohol, NAFLD, hepatitis B and C


# Investigations

- Bloods: Abnormal liver function tests often indicate autoimmune hepatitis, showing a hepatic pattern of disease, such as raised ALT and bilirubin with normal or mildly raised ALP. 
- Additionally, patients may present with an IgG predominant hypergammaglobulinemia. 
- The presence of specific antibodies helps to differentiate between the three types of autoimmune hepatitis:
	- Type I: Characterised by raised levels of anti-smooth muscle antibodies (80%) and possibly positive antinuclear antibodies (10%).
	- Type II: Less common and often more severe, typically positive for anti liver/kidney microsomal antibodies type 1.
	- Type III: Also less common, often positive for anti-soluble liver antigen.

# Management

* Management of autoimmune hepatitis is largely dependent on the severity of symptoms and the severity of disease, as determined by blood results and liver biopsy. 
* Initial treatment of acute episodes involves steroid (prednisolone) induction therapy, followed by maintenance therapy with azathioprine, which is effective in most cases. 
* For patients who do not respond to standard treatment, second-line treatment with other immunosuppressants can be considered.
* In patients presenting with chronic liver disease with decompensated cirrhosis or those who have failed to respond to immunosuppression, liver transplantation is the only management option.

# References

[British Society of Gastroenterology - Management of AIH](https://www.bsg.org.uk/clinical-resource/bsg-guidelines-for-the-management-of-autoimmune-hepatitis/)
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Autoimmune hepatitis
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Gastroenterology
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Autoimmune hepatitis
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Primary Biliary Cholangitis
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Autoimmune hepatitis
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Quesmed Tutorial: Liver Function Tests
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Anti-nuclear antibodies (ANA) are classically associated with Type I autoimmune hepatitis (AIH).
A 20-year-old female presents to the emergency department with fever and jaundice. Their LFTs are deranged, and their liver viral screen is negative. Autoimmune hepatitis (AIH) is suspected.

The presence of which of the following antibodies is classically associated with Type I AIH?
a