Biliary Disease Flashcards
Gallbladder bladder carcinoma is a rare carcinoma
True or false
True
What type of carcinoma is a gallbladder carcinoma
An adenocarcinoma
What is the strongest risk factor for gallbladder carcinoma
Gallstone disease
What kind of inflammation is associated with gallbladder carcinoma
Chronic inflammation
Which gallstone disease mostly causes a gallbladder carcinoma
A chronic gallstone disease leading to a porcelain gallbladder
What is the usual treatment for a patient with gallstone disease
Cholecystectomy
Which bacterial infection is also a risk factor for gallbladder carcinoma
Chronic salmonella infection (S. typhi)
What type of jaundice could a gallbladder carcinoma cause
Obstructive jaundice
How is a GC diagnosed
Ultrasound
What is the treatment for GC
Cholecystectomy (to remove the gallbladder and the cancer)
In general a GC has a ……… prognosis (good or poor)
Poor
What is a cholangiocarcinoma
An adenocarcinoma arising from bile duct epithelial cells
Can arise intra-hepatic or extra-hepatic
What are the extra-hepatic bile ducts
Common bile duct
Hepatic duct
Cystic duct
Which epithelial cells line the hepatic bile duct, the common bile duct and the cystic duct
Bile duct epithelial cells
What are the presenting symptoms of cholangiocarcinoma
Bile duct obstruction
Abdominal pain (RUQ)
Anorexia
Weight loss
Pruritus
Dark urine and clay-colored stools and jaundice
Most patients have no predisposing conditions
Which two key risk factors of cholangiocarcinoma could lead to chronic biliary inflammation
Primary sclerosing cholangitis (ulcerative colitis)
Clonorchis sinensis (Chinese liver fluke)
When a patient has things like jaundice and right upper quadrant pain, the first test that’s usually done is an RUG ultrasound because you’re investigating for conditions like …………. In which condition, do you have RUQ pain and jaundice but no sign of common duct stone or pancreatic cancer, especially when a patient has a risk factor like primary sclerosing cholangitis
A common bile duct stone or pancreatic cancer
How is a cholangiocarcinoma diagnosed
CT
MRI
MRCP
EUS (endoscopic ultrasound)
Depending on whether you think the lesion is intra or extra-hepatic
How are cholangiocarcinomas treated
Surgery
Radiation
Chemotherapy
Surgery only for localized disease
Poor prognosis
What is biliary cirrhosis
Liver damage secondary to biliary obstruction
Chronic obstruction of bile flow leads to liver damage
What are some things which could cause an obstruction to bile flow
Gallstones
Pancreatic cancer
Biliary strictures
Biliary strictures could be treated by
Stenting of bile ducts
What is the former name for primary biliary cholangitis
Primary biliary cirrhosis
What is primary biliary cholangitis
Biliary cirrhosis without extra-hepatic obstruction (no secondary cause of poor blood flow). It is an autoimmune disorder where T-cells attack on bile ducts which cause granules Marius inflammation. Most patients who have this condition have other autoimmune disorders as well
What size of bile ducts does a primary biliary cholangitis affect
Small to medium sized bile ducts
Primary biliary cholangitis is more common in which gender
Women
What are the most common initial symptoms of primary biliary cholangitis
Fatigue and pruritus
The fatigue in primary biliary cholangitis is often due to
Systemic inflammation
The pruritis in primary biliary cholangitis is often due to
The poor flow of bile into the intestines so bile acids and bile salts accumulate in the serum and they deposit in the skin which often causes intense itching, which may be severe especially at night (what often brings people to medical attention)
The pruritus in a primary biliary cholangitis often precedes development of jaundice so it is not always obvious initially that you’re dealing with a biliary disorder
True or false
True
How is primary biliary cholangitis diagnosed
Elevated alkaline phosphatase (usually to 3-5 times ULN (140 IU/L). In primary biliary cholangitis, alkaline phosphatase level 500+ are common
Mild elevations in the AST and ALT
Rise in bilirubin (associated with poor prognosis because it occurs late)
Anti-mitochondrial antibodies (present in 95% of patients)
Anti-nuclear antibodies seen in ~ 70% (non-specific)
Elevated serum lipids (TC > 1000 can be seen), xanthomas (corneal arcus) may occur
Imaging shows absence of biliary obstruction (because in this condition biliary obstruction is intra-hepatic not extra-hepatic)
Liver biopsy (not often done)
What test is the hallmark of primary biliary cholangitis
Anti-mitochondrial antibodies
What is the gold standard for a primary biliary cholangitis (PBC)
Liver biopsy
Woman with itching and fatigue
LFT shows markedly elevated Alk Phos
Positive anti-mitochondrial antibodies
What condition may this be
Primary biliary cholangitis
How is primary biliary cholangitis treated
Ursodeoxycholic acid
Liver transplant
What is the initial therapy for all patients with primary biliary cholangitis
Ursodeoxycholic acid
Similar to other bile acids but less toxic to hepaticytes
Replaces endogenous bile acids
Improves LFTd and disease progression
Treatment for PBC
What treatment could this be
Ursodeoxycholic acid
What is the most common associated disorder of PBC
Sjögren’s syndrome
What are some common associated disorders of PBC
Sjögren’s syndrome
Osteoporosis (poorly understood mechanism, serum calcium usually normal)
What is primary sclerosing cholangitis
It is an autoimmune disorder which causes inflammation, fibrosis and especially strictures in the biliary tree. This is going to obstruct the flow of bile from the liver into the intestines and cause signs and symptoms of biliary obstruction. It involves both intra and extra-hepatic bile ducts
How is primary sclerosing cholangitis differentiated from primary biliary cholangitis
Primary sclerosing cholangitis involves both the intra and extra-hepatic bile ducts. Biliary cholangitis is limited to the liver whereas in sclerosing cholangitis much of the problem is outside the liver in the common bile ducts and other elements of the biliary tree. Those conduits because obstructed by strictures
Primary sclerosing cholangitis is strongly associated with
Ulcerative colitis
What are the clinical features of PSC
Related to strictures that obstruct bile flow
Symptoms of biliary obstruction (RUQ pain, fatigue, jaundice, pruritus)
Often asymptomatic
Identified by abnormal LFTs on routine labs
The hallmark lab finding for PSC is evidence of what condition
Cholestasis
What are the lab findings in PSC
Elevated alkaline phosphatase
Elevated conjugated bilirubin
Usually mildly elevated AST/ALT
When cholestasis on their lab testing identified (listed above), patients are usually referred for biliary imaging and that’s how diagnosis is made
Besides laboratory evidence of cholestasis:
Positive p-ANCA antibodies (up to 80% in patients - also seen in UC)
Elevated IgM levels (up to 50% of patients)
Associated with HLA-DRw53a
How do you diagnose PSC
Suspected from cholestasis, especially in patients with ulcerative colitis
Ultrasound (often non-diagnostic, may show abnormal bile ducts - gallstone in the common bile duct or a pancreatic tumor)
Cholangiogram (can be done endoscopically through an ERCP or can be done as an MRCP - MRI version of cholangeography - tests shows strictures and dilatations (beading))
Liver biopsy (rarely diagnostic)
In which biliary disease is liver biopsy often diagnostic
Primary biliary cirrhosis
What imaging confirms the diagnosis in a PSC
Cholangiogram
Why is liver biopsy rarely diagnostic in a PSC
The problem is in the bile ducts especially the extra-hepatic bile ducts so in liver biopsy you rarely get a diagnostic result
How is PSC treated
Endoscopic therapy (dilatation or stenting of strictures in bile ducts)
Liver transplant if patients develops a cirrhosis from recurrent biliary obstructions
Patients with which conditions need regular screening for cholangiocarcinoma
Primary sclerosing cholangitis
*These patients have chronic inflammation of the bile duct epithelial cells and that puts them at high risk for cholangiocarcinoma
Which tumor marker is measured to check for primary sclerosing cholangiocarcinoma
CA 19-9
