Definition

[1]
Autoimmune Hepatitis (also AIH) is a chronic inflammatory liver condition caused by antibodies that attack hepatocytes.

Prevalence

[2]
17 per 100 000 people

Age

10-30, 40-60

Gender

Females are 3.6 times more likely

Risk Factors

[3]
  • Female
  • Family History
  • Other autoimmune conditions
  • Liver infection (weak)

Signs and Symptoms

[4]

Pathophysiology

[5]
The exact trigger of autoimmune hepatitis is not understood. Like many autoimmune conditions there are genetic and environmental factors that contribute to the development of the disease but many are not known. It is thought that T-cells attack hepatic auto-antigens which causes necrosis and fibrotic changes that impair the liver's functional capacity. This contributes to the classic signs of Chronic Liver Failure.

Investigations

[6] [7]
Bloods
  • Liver Function Tests - elevated or mildly elevated, low albumin
  • Serum globulin - polyclonal hypergammaglobulinaemia
  • Full Blood Examination - anaemia, thrombocytopaenia, raised white cell count
  • Anti-Nuclear Antibody - positive
  • Anti-Smooth Muscle Antibody - positive

Other

Liver Biopsy - interface hepatitis

Diagnosis

[8]
Diagnosis is based on hyper-IgG, autoantibodies and/or liver biopsy

Classification

[9]
  • Type I (80%) - Anti-smooth muscle antibody, sometime Anti-Nuclear Antibody, Hyper IgG
  • Type II - Anti-liver/ Antikidney microsomal type 1 antibodies, Anti-smooth muscle antibodies
  • Type III - Anti- soluble liver antigen and/or anti-liver-pancreas antigen, negative for ANA and ASMA

Differentials to rule out

[10]

Treatment

[11] [12]
Non-Pharmacological
  • Observation and monitoring (if non-severe)

Pharmacological

Surgical

  • Liver transplantation

Management

[13] [14]
Patients should be followed up monthly for progression of liver function tests, response to treatment and development of side effects.

Complications

[15]

Prognosis

[16] [17]
Without treatment the 5-year survival is <50%. With treatment more than 80% of patients are in remission after 18 months with 10 year survival >80%.

See Also

Oxford Handbook of Clinical Medicine - 9th edition p268
BMJ Best Practice - Autoimmune Hepatitis
  1. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  2. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  3. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  4. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  5. ^ DynaMed - Autoimmune Hepatitis
  6. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  7. ^ Oxford Handbook of Clinical Medicine - 9th edition p268
  8. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  9. ^ Oxford Handbook of Clinical Medicine - 9th edition p268
  10. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  11. ^ Oxford Handbook of Clinical Medicine - 9th edition p268
  12. ^ Oxford Handbook of Clinical Medicine - 9th edition p268
  13. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  14. ^ Oxford Handbook of Clinical Medicine - 9th edition p268
  15. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  16. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/130.html
  17. ^ Oxford Handbook of Clinical Medicine - 9th edition p268