AI Hepatitis

Question 1

What is AI hepatitis?

Answer 1

Chronic hepatitis of unknown aetiology, characterised by auto-antibodies, hyperglobulinaemia (increased Ig) + inflammatory changes on liver histology

Question 2

Describe the aetiology of AI hepatitis

Answer 2

Unknown, combination of:
Genetic predisposition
Environmental trigger: viruses, drugs
Autoantibodies

Question 3

What are the 2 main types of AI hepatitis?

Answer 3

Type 1 (Classic)
ANA
ASMA
Anti-soluble liver antigen (anti-SLA)
Type 2
Antibodies to liver/kidney microsomes (ALKM-1)
Antibodies to liver cytosol antigen (ALC-1)

Question 4

Describe the epidemiology of AI hepatitis

Answer 4

Type 1: occurs in ALL age groups (mainly young women)

Type 2: generally occurs in girls + young women

Question 5

What 8 symptoms may a patient with AI hepatitis insidiously present with?

Answer 5

Malaise  
Fatigue  
Anorexia  
Weight loss  
Nausea  
Jaundice  
Amenorrhoea  
Epistaxis

Question 6

List signs of AI hepatitis

Answer 6

Stigmata of chronic liver disease (e.g. spider naevi, hepatomegaly, jaundice)
Ascites, oedema + hepatic encephalopathy are late features
Cushingoid features (round face, acne, hirsuitism, cutaneous striae) may be present even before administration of steroids

Question 7

How may AI hepatitis present?

Answer 7

Asymptomatically: incidental finding on LFTs
Insidiously
Acutely

Question 8

List 6 symptoms of acute AI hepatitis

Answer 8

Fever  
Anorexia 
Jaundice  
N + V /Diarrhoea 
RUQ pain  
Serum sickness (e.g. arthralgia, polyarthritis, maculopapular rash)

Question 9

What should be established in the history of a patient with AI hepatitis?

Answer 9

PMH/ FH of other AI conditions

Full hx to exclude viral/ alcoholic hepatitis

Question 10

Describe LFTs in AI hepatitis

Answer 10

High: AST, ALT, GGT, ALP + BR
Low: albumin (in severe disease)
Clotting: High PT (in severe disease)

Question 11

Describe bloods in AI hepatitis

Answer 11

Low Hb, platelets + WCC (if hypersplenism from portal HTN)

Question 12

What other investigations are performed to exclude other caused of liver disease?

Answer 12

Viral serology (hep B/C)
Urinary copper/caeruloplasmin (Wilson’s disease)
Ferritin + transferrin saturation (haemochromatosis)
a-1 antitrypsin (for deficiency)
Anti-mitochondrial antibodies (PBC)
US, CT or MRI of liver + abdomen: Visualise structural lesions
ERCP: To exclude PSC

Question 13

Why is a liver biopsy needed in AI hepatitis?

Answer 13

To establish a definitive dx + check whether hepatitis or cirrhosis

Question 14

Hypergammaglobulinaemia is common in AI hepatitis, this is the presence of what?

Answer 14

ANA, ASMA + Anti-LKM antibodies