Autoimmune Hepatitis

Question 1

Define Autoimmune hepatitis

Answer 1

Chronic inflammatory disease of the liver with unknown aetiology characterised by:

• Presence of circulating auto-antibodies
• High serum globulin concentration
• Inflammatory changes on liver histology
• Favourable response to immunosuppressive treatment

Question 2

What are the 2 types of Autoimmune hepatitis

Answer 2

Based on the type of antibodies:
Type 1: ANA, SMA
Type 2: anti-liver kidney microsomal-1

Question 3

Aetiology of Autoimmune hepatitis

Answer 3

Unknown
Genetic predisposition: HLA DR3 and DR4
Environmental triggers: measles, CMV, hepatitis, drugs (methyldopa, diclofenac, atorvastatin)
APECED syndrome (immune regulatory dysfunction)

Question 4

Epidemiology of Autoimmune hepatitis

Answer 4

Type 1 is more common (78% women)

Type 2 mainly affects children 2-14 (95% women)

Question 5

Symptoms of Autoimmune hepatitis

Answer 5

Asymptomatic and discovered by abnormal LFTs

Fatigue and malaise 
Anorexia
Jaundice 
Pruritus 
Arthralgia 
Nausea
Fever
Abdominal distension 
Amenorrhoea 
Epistaxis

Question 6

Symptoms of acute hepatitis presentation (Autoimmune hepatitis)

Answer 6

Fever
Anorexia
Jaundice
Nausea
Vomiting
Diarrhoea 
RUQ pain
Serum sickness

Question 7

Signs of Autoimmune hepatitis on examination

Answer 7

Hepatomegaly
Liver disease: palmar erythema | Dupuytren’s contracture | bruising | spider naevi etc.
GI bleeding -> bruises
Splenomegaly
Ascites
Encephalopathy
Cushing’s I’d: round face, cutaneous striae, Caen, hirsutism

Question 8

Investigations for Autoimmune hepatitis

Answer 8

ANAs: Positive (high titre >1:80)
SMAs: Positive (high titre >1:80) - type 1

LFTs + enzymes: AST and ALT raised, bilirubin raised, GGT and alk phos moderately raised
Albumin: reduced
Globulin: raised markedly
PT: prolonged
FBC: mild anaemia, reduced platelets + WCC
Viral serology: rule out viral hepatitis
Caeruloplasmin: rule out Wilson’s
Iron studies: rule out Haemochromatosis
Alpha-1 antitrypsin: rule out deficiency
AMAs: Rule out PBC

Abdominal USS: biliary dilatation

Liver biopsy: peripheral lesion or interface hepatitis, may see cirrhosis