Hepato-biliary: Autoimmune

Question 1

Sequence of events in autoimmune heptatits

Answer 1

Sequence of events:
•	White cells attack the liver
•	Chronic inflammation
•	Fibrosis
•	Necrosis

Question 2

3 types of autoimmune hepatitis (and their antibodies)

Answer 2

AIHI: ASMAs, ANAs
AIH2: LKM-1, anti LC1 (typically children)
AIH3: soluble liver proteins liver pancreas antigen

Question 3

Presenation autoimmune hepatitis

Answer 3

•	As chronic liver disease
o	Palmar erythema
o	Hepatomegaly
o	Splenomegaly 
o	Myalgia 

•	As acute hepatitis 
o	Jaundice
o	Abdominal pain 
o	N+V
o	Loss of appetite

Question 4

Investigations for autoimmune hepatitis

Answer 4

Blood Tests:
•	FBC- raised ESR, WCC
•	LFT’s (markers of liver damage) 
o	Bilirubin 
o	Aminotransferases (ALT + AST)

• Immunoglobulin’s
o Raised IgG

• Antibodies
o ASMA positive
o Antinuclear factor positive

Imaging:
• Liver Biopsy

Question 5

Management for autoimmune hepatitis

Answer 5

Medical:
• Steroids e.g. Prednisolone
• Immunosuppressant’s e.g. Azathioprine
• Ursodeoxycholic Acid

Surgical:
• Liver transplant

Question 6

What does PBC stand for?

Answer 6

Primary biliary cirrhosis

Question 7

Who does PBC typically affect>

Answer 7

Women, typically manifests following pregnancy

Question 8

Sequence of events in PBC

Answer 8

GRANULOMATOUS INFLAMMATION IN INTRAHEPATIC BILE DUCTS

Bile Duct Obstruction/Cholestasis

Cirrhosis

(T cells attack bile ducts, bile leaks out causing inflammation)

Question 9

Presentation of PBC

Answer 9

(similar to cholestasis and obstructive jaundice)
PRURITIS: With or without jaundice
Jaundice - conjugated hyperbilirubinaemia
Fatigue
Arthralgia
Xanthelasma

Question 10

Investigations for PBC

Answer 10

LFTs - bilirubin, alkp, GGT,

Immunoglobulins - raised IgM

Antibodies - AMA (Anti-Mitochondrial Antibodies) positive

Question 11

Management PBC

Answer 11

Steroids eg prednisolone

Immunosuppressant’s e.g. Azathioprine

Ursodeoxycholic Acid - Life long

Cholestyramine - Pruritus

Surgical:
• Liver transplant

Question 12

What is PBC associated with

Answer 12

Sjogren’s

RA

Question 13

Prognosis PBC

Answer 13

Asymptomatic - normal life expectancy

Symptomatic - 5 year survival

Question 14

What does PSC stand for?

Answer 14

Primary Sclerosing Cholangitis

Question 15

What is PSC associated with?

Answer 15

IBD - mainly UC

Question 16

Sequence of events in PSC

Answer 16

CHRONIC INFLAMMATION IN INTRAHEPATIC (+EXTRA) BILE DUCTS

Bile Duct

Obstruction/Cholestasis

Cells become inflamed, die and harden (tightening and dilating)

Question 17

Presentation PSC

Answer 17

May be asymptomatic - detected in UC patients with abnormal liver biochemistry

• Pruritus
• Jaundice - Conjugated hyperbilirubinaemia
• Cholangitis- fever, rigors, jaundice, abdominal pain
• Fatigue
• Weight loss

V. close to portal veins so can cause PHT - hepatosplenomegaly, cirrhosis

Question 18

Investigations for PSC

Answer 18

LFTs - bilirubin, alkp

Immunoglobulins - raised IgM

Antibodies - pANCA (myeloperoxidase antineutrophillic cytoplasmic antibodies) positive(ANA positive)

Imaging: ERCP/MRCP diagnosis

Question 19

Management of PSC

Answer 19

Immunosuppressant’s e.g. Methotrexate

Ursodeoxycholic Acid - May slow disease progression

Cholestyramine - Pruritus

Endoscopic - ERCP
 Sphincterectomy/stent insertion

Question 20

Complications of PSC

Answer 20

Cholangiocarcinoma

Sclerosing cholangitis seen in AIDS