Primary Sclerosing Cholangitis

Question 1

What is primary sclerosing cholangitis.

Answer 1

A progressive fibrosis and obliteration of the biliary tracts (both intra and extra hepatic).

Question 2

Who gets primary sclerosing cholangitis. (3)

Answer 2

70% male.
Aged 30-50.
Associated with IBD (mainly UC).

Question 3

What other condition is primary sclerosing cholangitis associated with.

Answer 3

Inflammatory bowel disease (mainly ulcerative colitis).

Question 4

What are the clinical features of primary sclerosing cholangitis. (8)

Answer 4

May be asymptomatic. 
Fatigue. 
Weight loss. 
Fever. 
Pruritus. 
RUQ discomfort. 
Hepatomegaly.
May have relapsing  ascending cholangitis.

Question 5

What does primary sclerosing cholangitis entail.

Answer 5

It entails progressive cholestasis with bile duct inflammation and strictures.

Question 6

What are the initial symptoms of primary sclerosing cholangitis. (2)

Answer 6

Pruritus.

Fatigue.

Question 7

What are the clinical signs of primary sclerosing cholangitis if it is advanced. (3)

Answer 7

Ascending cholangitis.
Cirrhosis.
End stage hepatic failure.

Question 8

What is associated with primary sclerosing cholangitis. (4)

Answer 8

Male.
HLA-A1, B8, DR3.
AIH.
IBD (mainly UC, often presents before PSC).

Question 9

What cancers are associated with PSC. (4)

Answer 9

Bile duct.
Gallbladder.
Liver.
Colon.

Question 10

What bile ducts are most affected in PSC.

Answer 10

Both the intra and extra hepatic.

Question 11

What percentage of patients with PSC also have UC.

Answer 11

75%

Question 12

What autoantibody is found in PSC cases.

Answer 12

pANCA.

Question 13

What percentage of patients with PSC have pANCA present.

Answer 13

60%

Question 14

What is the average age of onset for PSC.

Answer 14

40.

Question 15

What conditions can also have secondary PSC. (2)

Answer 15

HIV.

Cryptosporidium.

Question 16

How is the diagnosis of PSC made.

Answer 16

MRCP.

Question 17

What is seen histologically in patients with PSC. (2)

Answer 17

Inflammation of the intrahepatic biliary radicles.

Associated scar tissue (‘onion skin’ in appearance).

Question 18

What is the incidence of PSC.

Answer 18

6/100,000 in caucasians.

Question 19

What percentage of patients with PSC will go on to develop cholangiocarcinoma.

Answer 19

10-30%

Question 20

What is the diagnosis criteria for PSC. (3)

Answer 20

Generalised beading and stenosis of the biliary system on cholangiography.
Absence of choladocholithiasis (or history of bile duct surgery).
Exclusion of bile duct cancer.

Question 21

What are some causes of secondary PSC. (8)

Answer 21

Previous bile duct surgery with stricturing and cholangitis.
Bile duct stones causing cholangitis.
Intrahepatic infusion of 5-fluorodeoxyuridine.
Insertion of formalin into hepatic hydatid cysts.
Insertion of alcohol into hepatic tumours.
Parasitic infection.
Autoimmune pancreatitis.
Acquired immunideficiency syndrome (AIDS).

Question 22

What diseases are associated with PSC. (12)

Answer 22

UC. 
Crohn's colitis. 
Chronic pancreatitis. 
Retroperitoneal fibrosis. 
Riedel's thyroditis. 
Retro-orbital tumours. 
Immune deficiency states. 
Sjogren's syndrome. 
Angio-immunoplastic lymphadenopathy. 
Histiocytosis X. 
Autoimmune haemolytic anaemia. 
Autoimmune pancreatitis.

Question 23

How is the diagnosis usually made for PSC.

Answer 23

Incidentally when persistently raised serum ALP is discovered in an individual with UC.

Question 24

What is the key investigation now used to diagnose PSC.

Answer 24

MRCP.

Question 25

What are the blood results in a patient presenting with PSC. (8)

Answer 25

Raised ALP. 
Raised bilirubin. 
Hypergammaglobulinaemia. 
ANCA positive. 
ANA positive. 
SMA positive.
AMA negative.