primary sclerising cholangitis Flashcards
primary sclerosing cholangitis
chronic cholestatic liver disease
characterised by progressive inflammatory fibrosis and obliteration of intrahepatic and extrahepatic bile ducts
bile duct strictures
aetiology of primary sclerosing cholangitis
unknown
postulated immune and genetic predisposition
toxic and infective triggers
associations with primary sclerosing cholangitis
close association with IBD, especially UC (present in 70%)
about 5% of those with UC -> develop PSC
combination of PSC and UC = increased risk of colorectal malignancy
HLA-A1, HLA-B8, HLA-DR3
autoimmune hep (AIH)
epidemiology of primary sclerosing cholangitis
prevalence 2-7 in 100000
usually presents between 25-40yrs
male more
sx of primary sclerosing cholangitis
asymptomatic - dx after persistently high ALP
intermittent jaundice, pruritis, RUQ pain, weight loss, fatigue
episodes of fever and rigors caused by acute cholangitis are less common
history of UC
sx of complications
signs of primary sclerosing cholangitis
may have no signs
jaundice
hepatosplenomegaly
spider naevi
palmar erythema
ascites
if advanced signs of - ascending cholangitis, cirrhosis and hepatic failure
pathology of primary sclerosing cholangitis
periductal inflammation with periductal concentric fibrosis (onion skin)
portal oedema
bile duct proliferation
expansion of portal tracts
progressive fibrosis
development of biliary cirrhosis
ix for primary sclerosing cholangitis
blood
serology
ERCP
MRCP - non-invasive imaging of the biliary tree
liver biopsy - confirms dx and allows staging of the disease - shows fibrous, obliterative cholangitis
blood results for primary sclerosing cholangitis
LFT - high ALP,. high GGT, mildly high transaminases.
LFT in later stage - low albumin and high BR
hypergammaglobinaemia and/or high IgM
AMA -ve
ANA, SMA and ANCA mnay be +ve
serology for primary sclerosing cholangitis
Ig - high IgG, high IgM
ASM and ANA present in 30%
AMA absent
pANCA - present in 70%
ERCP result for primary sclerosing cholangitis
stricturing and interspersed dilation (beading) of intrahepatic and occaisionaly extrahepatic bile ducts
small diverticula on common bile duct may be seen
ie shows duct anatomy and damage
what does primary sclerosing cholangitis predispose to
cancers:
- bile duct
- gallbladder
- liver
- colon
yearly colonoscopy + US
consider cholecystectomy for gallbladder polyps
mx of PSC
asx = monitoring + lifestyle
* oesteoporosis
* ileocolonoscopy every 5yrs
if itch - rule out strictures with MRCP
* if fine -> antipuriritic eg cholestyramine
consider ursodeoxycholic acid
liver transplant
complications of PSC
- ascites
- oesophageal varices
- hepatic encephalopathy
- hepatocellular cancer
- cholangiocarcinoma
- osteoporosis
- fat sol vit def
- bacterial cholangitis
- choledocholithiasis
- gallbladder ca
- colon ca
monitoring for PSC
LFTs
liver elastography/serum fibrosis every 3yrs
DEXA
test for vit def
Ab for Hep A or B and offer vaccine if needed
ileocolonoscopy
