Primary sclerosing cholangitis Flashcards
Define primary sclerosing cholangitis
Chronic cholestatic liver disease characterised by progressive fibrosis & obliteration of intrahepatic & extra hepatic bile ducts
Aetiology of primary sclerosing cholangitis
3
UNKNOWN
Possible immune & genetic predisposition w/ environmental triggers
Close association w/ IBD (UC especially - present in 70% PSC patients)
Pathogenesis of primary sclerosing cholangitis
6
Periductal inflammation w/ periductal concentric fibrosis Portal oedema Bile duct proliferation Expansion of portal tracts Progressive fibrosis Development of biliary cirrhosis
Epidemiology of primary sclerosing cholangitis
age
Usually presents at 25-40 yrs old
Presenting symptoms of primary sclerosing cholangitis
7
May be asymptomatic & diagnosed after persistently raised ALP
May present with: intermittent jaundice pruritus RUQ pain weight loss fatigue
May present w/ episodes of fever & rigors caused by acute cholangitis (less common)
Signs of primary sclerosing cholangitis on physical examination
(6)
May have no signs Jaundice Hepatosplenomegaly Spider naevi Palmar erythema Ascites
Investigations for primary sclerosing cholangitis
5
Bloods Serology ERCP MRCP Liver biopsy
Investigations for primary sclerosing cholangitis - bloods
LFTS
high ALP & GGT
mildly elevated ALT & AST
low albumin & high bilirubin (later stages)
Investigations for primary sclerosing cholangitis - serology
(5)
IgG high in children IgM high in adults ASMA & ANA present in 30% AMA usually ABSENT Perinuclear anti neutrophil cytoplasmic antibodies (pANCA) present in 70%
Investigations for primary sclerosing cholangitis - ERCP
2
Shows stricturing & interspersed dilation of intrahepatic & (occasionally) extrahepatic bile ducts
Small diverticuli on common bile duct may be seen
Investigations for primary sclerosing cholangitis - MRCP
Enables non-invasive imaging of the biliary tree
Investigations for primary sclerosing cholangitis - liver biopsy
Confirms diagnosis & allows staging
