Autoimmune Hepatitis Flashcards

1
Q

What is autoimmune hepatitis (AIH)?

A

A chronic inflammatory disease of the liver of unknown aetiology. It is characterised by the presence of circulating auto-antibodies with a high serum globulin concentration, inflammatory changes on liver histology and a favourable response to immunosuppressive treatment.

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2
Q

Briefly descibre the aetiology of AIH

A
  1. Genetic predisposition
  2. Environmental triggers (e.g. drugs, viral infections or herbal agents)
  3. Auto-antigens
  4. Dysfunction of immunoregulatory mechanisms
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3
Q

What are the risk factors for AIH?

A
  • Female gender
  • Genetic predisposition
  • Immune dysregulation
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4
Q

What are the signs of AIH?

A
  • Hepatosplenomegaly
  • Jaundice
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5
Q

What are the symptoms of AIH?

A
  • Fatigue and malasie
  • Anorexia
    *
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6
Q

What investigations should be ordered for AIH?

A
  • AST level
  • ALT level
  • Bilirubin
  • Gamma glutamyl transferase (Gamma-GT)
  • Alkaline phosphatase
  • Serum globulin
  • Serum albumin
  • Prothrombin time
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7
Q

Why investigate AST? And what may this show?

A
  • Indicator of inflammatory activity in the liver. Raised in 100% of patients with AIH at initial presentation.
  • Raised in other liver diseases. Not specific to liver disease.
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8
Q

Why investigate ALT? And what may this show?

A
  • Indicator of inflammatory activity in the liver. Raised in 100% of patients with AIH at initial presentation.
  • Raised in other liver diseases. Not specific to liver disease.
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9
Q

Why investigate bilirubin? And what may this show?

A
  • Increased in 80% to 90% of patients with AIH
  • Mild to moderately increased
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10
Q

Why investigate gamma-GT? And what may this show?

A
  • Mild to moderately increased
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11
Q

Why investigate alkaline phosphatase? And what may this show?

A
  • Mild to moderately increased
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12
Q

Briefly describe the treatment for AIH

A

Corticosteroids (e.g. prednisolone) and immunosuppressants (e.g. azathioprine) form the mainstay of treatment.

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13
Q

Give examples of patients in which corticosteroids should be used with caution

A

Patients with:

  • Cirrhosis
  • Post-menopausal osteopenia or vertebral compression
  • Emotional lability or psychosis
  • Poorly controlled hypertension
  • Brittle diabetes are at increased risk of adverse effects with corticosteroids
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14
Q

When is liver transplantation considered in AIH?

A

Indicated in patients who deteriorate during or after corticosteroid treatment and in patients who are refractory to or intolerant of standard treatment and in whom end-stage liver disease develops.

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15
Q

What complications are associated with AIH?

A
  • Osteoporosis due to corticosteoid therapy
  • Diabetes mellitus due to corticosteroid therapy
  • Hypertension due to corticosteroid therapy
  • Truncal obesity due to corticosteroid therapy
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16
Q

What differentials should be considered for AIH?

A
  1. Primary biliary cirrhosis
  2. Primary sclerosing cholangitis
17
Q

How does AIH and primary biliary cirrhosis differ?

A
  • Differentiating signs and symptoms: main complaints are fatigue and pruritus, which are usually more pronounced than in AIH
  • Differentiating investigations:
    • Alkaline phosphatase and gamma-GT raised more strikingly than aminotransferase levels.
    • Anti-mitochondrial antibodies (AMAs) present in 95% of cases and antinuclear antibodies (ANAs) in up to 70%
    • Liver biopsy shows bile duct lesion or periductal fibrosis
18
Q

How does AIH and primary sclerosing cholangitis differ?

A
  • Differentiating signs and symptoms:
    • Features often overlap with AIH
    • More common in men than in women.
    • Strongly associated with inflammatory bowel disease
  • Differentiating investigations:
    • Markedly raised alkaline phosphatase
    • Abnormal cholangiogram
    • Liver biopsy shows bile duct abnormalities
    • No response to corticosteroids