Biliary/Pancreatic Disease Flashcards
Primary sclerosing cholangitis is
a biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts.
Primary sclerosing cholangitis associations?
ulcerative colitis
Crohn’s (much less common association than UC)
HIV
?% of patients with UC have PSC, ?% of patients with PSC have UC
4% of patients with UC have PSC, 80% of patients with PSC have UC
PSC bloods
raised bilirubin + ALP
PSC sx
cholestasis
jaundice, pruritus
right upper quadrant pain
fatigue
PSC ix
endoscopic retrograde cholangiopancreatography (ERCP)
or magnetic resonance cholangiopancreatography (MRCP)
are the standard diagnostic investigations, showing multiple biliary strictures giving a ‘beaded’ appearance
PSC which ANCA might be positive
p-ANCA may be positive
PSC always use liver biopsy
false
limited role for liver biopsy,
PSC what would liver biopsy show
fibrous, obliterative cholangitis often described as ‘onion skin’
PSC complications
cholangiocarcinoma (in 10%)
increased risk of colorectal cancer
Primary biliary cholangitis is a chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1)
true
Primary biliary cholangitis aetiology is not fully understood although it is thought to be an autoimmune condition
true
Primary biliary cholangitis classic presentation
itching in a middle-aged woman
Primary biliary cholangitis pathophysiology?
Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis
Primary biliary cholangitis associations
Sjogren’s syndrome (seen in up to 80% of patients)
rheumatoid arthritis
systemic sclerosis
thyroid disease
PBC sx
early: may be asymptomatic or fatigue, pruritus
cholestatic jaundice
hyperpigmentation, especially over pressure points
around 10% of patients have right upper quadrant pain
xanthelasmas, xanthomata
also: clubbing, hepatosplenomegaly
late: may progress to liver failure
PBC LFTs
raised ALP on routine LFTs
PBC diagnosis?
anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific
smooth muscle antibodies in 30% of patients
raised serum IgM
PBC mx
first-line: ursodeoxycholic acid
slows disease progression and improves symptoms
pruritus: cholestyramine
fat-soluble vitamin supplementation
PBC liver is transplanted is indicated when?
if bilirubin > 100 (PBC is a major indication)
recurrence in graft can occur but is not usually a problem
PBC complications
cirrhosis → portal hypertension → ascites, variceal haemorrhage
osteomalacia and osteoporosis
significantly increased risk of hepatocellular carcinoma (20-fold increased risk)
Viral hepatitis common symptoms include:
nausea and vomiting, anorexia
myalgia
lethargy
right upper quadrant (RUQ) pain
Viral hepatitis Questions may point to risk factors such as
foreign travel or intravenous drug use.