Autoimmune Hepatitis

almostadoctor.co.uk/encyclopedia/autoimmune-hepatitis

Dr Tom Leach

Introduction
Autoimmune hepatitis (AIH) is a cause of chronic hepatitis. It is also sometimes referred
to as chronic active hepatitis. Any hepatitis that typically lasts >6 months is referred to
as chronic.
It is characterised by:
Increased IgG levels
Antibodies against:
Liver specific proteins
Non-live specific proteins
Mononuclear infiltrate within the liver
Monocytes, macrophages, lymphocytes, plasma cells, macrophages and mast
cells

There are three types of autoimmune hepatitis, which can be distinguished histologically:
AIH – 1 – shows the presence of anti-smooth muscle antibodies (ASMA’s),
andsometimes anti-nucelar antibodies (ANA’s)
AIH – 2 – has liver-kidney microsomal type 1 antibodies (LKM-1) and
sometimes anti-liver cytosol 1 antibodies (anti-LC1).
AIH – 3 – has antibodies to soluble liver proteins or liver-pancreas antigen.
Actin antibody is related to prognosis. Those with actin antibody presence
are more likely to require transplant than those without.
HLA-DR3 and HLA-DR4 are associated with increased risk for AIH.
In HLA-DR3 patients the disease tends to occur earlier, and is more likely to
require transplantation.
ANA’s – antinuclear antibodies are present in many autoimmune
diseases: Rheumatoid arthritis, SLE, scleroderma, polymyositis, dermatomyositis,
as well as AIH. In many of these diseases the ANA blood test can help
diagnosis.

Epidemiology
Autoimmune hepatitis is rare. In Western Europe and the US there are <1 case per
100 000
The M:F ratio is 1:3
Peaks of incidence: Age 10-20, and 45-70. The lower peak is more common and:
§ Accounts for about half of cases
§ Typically premenstrual girls
§ 80% of AIH-2 patients are children

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 Other autoimmune disease in the patient, or first degree relatives:
20% of patients have another autoimmune disease
40% of patients have a first degree relative with an autoimmune disease

Pathology
The disease is thought to occur in genetically pre-disposed individuals who come into
contact with an environmental trigger.
Many patients with autoimmune hepatitis have a low CD8 count. They also often have a
genetically determined reduced level of CD4 T cells, which are associated with fighting
viral infection. Thus it may be possible that a viral infection triggers off the production of
autoantibodies in susceptible individuals. For example, the hepatitis C virus has a close
relation to LKM-1 antibody mentioned above.

Drugs and Environmental agents have also been cited as possible causes.

Very rarely, AIH may be the result of Hep A, Hep B, EBV

More commonly AIH is caused by Hep C

Presentation
May initially be acute presentation, but will always eventually become chronic.
Up to 50% of patients present as if an acute viral hepatitis.
In other cases, there is typically an insidious onset
Non-specific symptoms:
Fatigue
Anorexia
Weight loss
Amenorrhoea
Behavioural changes
Abdominal pain
Liver-specific problems:
Epistaxis – nose bleeds
Easy bruising
Jaundice
Hepatomegaly / splenomegaly (50-60% of cases)
Ascties
Signs associated with chronic liver disease: Asterixis, Palmar erythema, Spider
naevi

Prognosis
Many patients will have severe cirrhosis by the time of diagnosis
About 25% of patients will have a liver transplant

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