Gastro: Primary billiary/sclerosing cholangitis Flashcards
Conditions associated with primary billiary cholangitis?
Sjogren’s syndrome (seen in up to 80% of patients)
Rheumatoid arthritis
Systemic sclerosis
Thyroid disease
“Classic” presentation of primary billiary cholangitis
Itching in a middle-aged woman.
Clinical features of primary billiary cholangitis?
Early: may be asymptomatic (e.g. raised ALP on routine LFTs) or fatigue, pruritus
Cholestatic jaundice
Late: may progress to liver failure
Bloods classically raised in primary billiary cholangitis?
Anti-mitochondrial antibodies (AMA) highly specific
Smooth muscle antibodies in 30% of patients
Raised serum IgM
Management of primary billiary cholangitis?
Pruritus: cholestyramine
Fat-soluble vitamin supplementation
Ursodeoxycholic acid
Liver transplantation e.g. if bilirubin > 100
What ducts are affected primary biliary cholangitis vs primary sclerosing cholangitis?
PBC: intrahepatic
PSC: intra and extra hepatic
What condition is commonly associated with primary sclerosing cholangitis
Ulcerative colitis
What are patients with PSC at risk of?
cholangiocarcinoma - lifetime risk of 5-15%.
Symptoms of PSC?
Early: fatigue, pruritus, right upper quadrant pain, jaundice
Later: cirrhosis, portal hypertension, and cholangiocarcinoma
Bloods raised in PSC?
elevated ALP and GGT
p-ANCA may be positive
PSC classic findings on ERCP/MRCP?
multiple biliary strictures giving a ‘beaded’ appearance
Management PSC
Pruritus: cholestyramine
Ursodeoxycholic acid.
Endoscopic dilation/stenting
A liver transplant is the only definitive treatment for advanced disease
How does ursodeoxycholic acid work to improve prognosis in PBC vs PSC
PBC improves survival
PSC no affect on survival but may improve LFTs
