Primary Sclerosing Cholangitis

Question 1

Define Primary Sclerosing Cholangitis

Answer 1

Chronic progressive cholestatic liver disease characterised by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts that results in diffuse multi-focal stricture formation

Question 2

Aetiology of Primary Sclerosing Cholangitis

Answer 2

Unknown
Associated with autoimmune disease, Inflammatory bowel disease (80% of PSC have UC), HLA haplotypes
Predominant in males

Stricture formation -> stenosis -> obstruction + progressive fibrosis -> ductopenia -> cholestasis

Question 3

Symptoms of Primary Sclerosing Cholangitis

Answer 3

Abdominal pain (RUQ/epigastric)
Pruritus (generalised, intermittent)
Jaundice
Fatigue 
Weight loss (Fat malabsorption)
Fever
Steatorrhoea

Question 4

Signs of Primary Sclerosing Cholangitis on examination

Answer 4

Jaundice
Portal HTN: splenomegaly, ascites

Encephalopathy

Question 5

Investigations for Primary Sclerosing Cholangitis

Answer 5

Serology: p-ANCA
MRCP: Normal O)R multi-focal intrahepatic and/or extrahepatic stricutres + dilations
ERCP: Normal or multi-focal strictures and dilations - shows a “beaded appearance”

LFTs: GGT and Alk phos ↑↑ | AST/ALT ↑ | BIlirubin ↑ | Albumin normal/low
FBC: normal OR thrombocytopenia ± anaemia, leukopenia
Clotting: PT normal/prolonged
Serology: AMA/ASMA/ANA negative
Serum immunoglobulins: IgM elevated
Caeruloplasmin: elevated
Hepatic/urine copper concentration: elevated

Abdominal USS: abnormal bile duct ± cirrhotic liver, ascites, splenomegaly
CT: bile duct thickening, duct dilation, lymphadenopathy

Liver biopsy: Periductal fibrosis and inflammation + ducts obliterated
Colonoscopy: Screen for IBD