Primary Sclerosing Cholangitis Flashcards
What is primary sclerosing cholangitis.
A progressive fibrosis and obliteration of the biliary tracts (both intra and extra hepatic).
Who gets primary sclerosing cholangitis. (3)
70% male.
Aged 30-50.
Associated with IBD (mainly UC).
What other condition is primary sclerosing cholangitis associated with.
Inflammatory bowel disease (mainly ulcerative colitis).
What are the clinical features of primary sclerosing cholangitis. (8)
May be asymptomatic. Fatigue. Weight loss. Fever. Pruritus. RUQ discomfort. Hepatomegaly. May have relapsing ascending cholangitis.
What does primary sclerosing cholangitis entail.
It entails progressive cholestasis with bile duct inflammation and strictures.
What are the initial symptoms of primary sclerosing cholangitis. (2)
Pruritus.
Fatigue.
What are the clinical signs of primary sclerosing cholangitis if it is advanced. (3)
Ascending cholangitis.
Cirrhosis.
End stage hepatic failure.
What is associated with primary sclerosing cholangitis. (4)
Male.
HLA-A1, B8, DR3.
AIH.
IBD (mainly UC, often presents before PSC).
What cancers are associated with PSC. (4)
Bile duct.
Gallbladder.
Liver.
Colon.
What bile ducts are most affected in PSC.
Both the intra and extra hepatic.
What percentage of patients with PSC also have UC.
75%
What autoantibody is found in PSC cases.
pANCA.
What percentage of patients with PSC have pANCA present.
60%
What is the average age of onset for PSC.
40.
What conditions can also have secondary PSC. (2)
HIV.
Cryptosporidium.
How is the diagnosis of PSC made.
MRCP.
What is seen histologically in patients with PSC. (2)
Inflammation of the intrahepatic biliary radicles.
Associated scar tissue (‘onion skin’ in appearance).
What is the incidence of PSC.
6/100,000 in caucasians.
What percentage of patients with PSC will go on to develop cholangiocarcinoma.
10-30%
What is the diagnosis criteria for PSC. (3)
Generalised beading and stenosis of the biliary system on cholangiography.
Absence of choladocholithiasis (or history of bile duct surgery).
Exclusion of bile duct cancer.
What are some causes of secondary PSC. (8)
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).
What diseases are associated with PSC. (12)
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.
How is the diagnosis usually made for PSC.
Incidentally when persistently raised serum ALP is discovered in an individual with UC.
What is the key investigation now used to diagnose PSC.
MRCP.
What are the blood results in a patient presenting with PSC. (8)
Raised ALP. Raised bilirubin. Hypergammaglobulinaemia. ANCA positive. ANA positive. SMA positive. AMA negative.
