Flashcards in Biliary tract diseases Deck (64):
What is Primary Biliary Cholangitis?
An autoimmune condition causing granulomatous inflammation
There is progressive destruction of intrahepatic bile ducts
This leads to cholestasis
Which leads to cirrhosis and portal hypertension
What are the intrahepatic bile ducts? Where are they?
They are small ducts that run throughout the liver
Transporting bile produced in the hepatocytes through the liver to the larger ducts that take the bile to the gall bladder
Describe the journey of bile from its production to its storage to its release into the small intestine?
Produced by hepatocytes
Released into bile canaliculi
Into interlobular bile ducts
Into intrahepatic bile ducts
Released into the R or L hepatic duct
These merge to form the common hepatic duct
Goes into the cystic duct to reach the gall bladder
Stored in gall bladder
Released from gall bladder back down cystic duct
Goes down common bile duct into the duodenum
What is cholestasis?
When bile can't flow from the liver to the gall bladder so it builds up and, along with other toxins, causes damage to the liver
Which people are most often affected with PBC?
Women between age 40-50
What is the difference between Primary Biliary Cirrhosis and Primary Biliary Cholangitis?
They are the same!
Primary Biliary Cholangitis is the new name for it
What causes PBC?
But the disease is set off in these people by an environmental trigger
Such as infection or pollution
What are the risk factors of developing PBC?
Having other autoimmune disease
What are the clinical features of PBC?
Often found incidentally via LFT blood tests
Signs of liver failure:
- hepatic encephalopathy
What is Xanthoma?
Deposition of yellowish cholesterol-rich material that can appear anywhere in the body
These can appear on the skin as yellow blob-like lesions
What 2 types of cholestasis are there?
Hepatocellular: the hepatocytes aren't making bile
Obstructive: something is blocking the flow of bile
What type of cholestasis is PBC?
What are the complications of PBC?
- Portal hypertension
- Liver failure
Malabsorption of fat soluble vitamins
Which are the fat soluble vitamins?
A, D, E, K
Why does PBC cause malabsorption of fat soluble vitamins?
Because bile is essential for the digestion of lipids
So if no bile, no lipid absorption
So fat soluble vitamins can't be absorbed
Why does PBC cause coagulopathy?
Because PBC causes liver damage and cirrhosis
This impairs the liver's ability to make clotting proteins
Fewer clotting proteins = less ability of blood to clot
Investigations of PBC?
- raised serum alkaline phosphate
- in late disease, raised bilirubin + low albumin
- exclude extra-hepatic cholestasis: problems with gall bladder or pancreas
- not usually needed, but look for granulomata round bile ducts
- look for cirrhosis + scarring
Management of PBC?
Treat the symptoms: pruritus, diarrhoea, osteoporosis
Give fat soluble vitamin supplements to accommodate malabsorption
Drug treatment: UDCA
Regular monitoring of liver function and USS
What is UDCA?
It reduces ascites, jaundice and can improve survival and delay the need for transplant
What is the prognosis of PBC?
Once jaundice develops, less than 2 years
What is Primary Sclerosing Cholangitis?
Progressive obliteration, inflammation and narrowing of intra + extra-hepatic ducts
This leads to cholestasis
Eventually leading to strictures, cirrhosis and gallstones
What is the difference between PBC and PSC?
PBC: only affects intra-hepatic ducts, no strictures or gallstones. affects women more
PSC: affects intra and extra-hepatic ducts, strictures and gallstones, strong links with cancer, affects men more
Which one of PBC and PSC is linked with inflammatory bowel disease?
Over 50% have both
Clinical presentation of PSC?
Pain + rigors
Signs of liver damage
What are the risk factors for PSC?
Also having IBD, most commonly UC
What are the complications of PSC?
Complications associated with this: portal hypertension, encephalopathy
- gall bladder adenocarcinoma
What is cholangiocarcinoma?
Bile duct cancer
Investigations of PSC?
- raised alkaline phosphate
- raised bilirubin
- ANCA (autoantibody)
- do check for extra + intra-hepatic bile duct involvement
- shows cholangitis: fibrous scarred bile ducts
What is ANCA?
Anti-neutrophil cytoplasmic antibody
An autoantibody, linked with autoimmune disease
Why is it important to do an ERCP on patients with suspected PSC?
Distinguishes between PSC and PBC
ERCP will show up whether there is extra-hepatic bile duct involvement
If there is suspect PSC
If not suspect PBC
Management of PSC?
UDCA drug: may improve liver function and protect against colon cancer
Treat symptoms: pruritus
Screen regularly for cancers of bile duct, gall bladder, liver, colon
What is biliary colic?
Pain related to the gall bladder associated with the temporary obstruction of the cystic duct or common bile duct
Usually caused by gallstones
Obstruction of which ducts in the biliary system causes biliary colic?
Where are they?
Cystic duct: from gall bladder to
common bile duct
Common bile duct: from joining of cystic duct to duodenum
What are the 2 types of gallstones?
Which are more common?
Cholesterol: most common - 80%
Why do cholesterol gallstones form?
In bile with excess cholesterol
In gall bladders with reduced motility
What are pigment gallstones made of and why do they form?
Bilirubin polymers and other bilirubin compounds
So they are seen in people who have high levels of bilirubin:
- sickle cell disease
What are the risk factors for developing cholesterol gall stones?
Multiparity: given birth to many children
High fat diet
Investigations of gallstones?
Evidence from history
- increased serum alkaline phosphate
- increased bilirubin
- inflammatory features could indicate cholecystitis
What problems can gallstones cause?
Cholecystitis: inflammation of gall bladder
Cholangitis: inflammation of bile ducts
How do gallstones cause pancreatitis?
A stone can block the common bile duct or even move into the pancreatic duct
Blocking pancreatic enzymes from leaving the pancreas means they build up and become toxic, damaging the pancreas
What are the clinical features of gallstones?
Severe right upper quadrant pain
Pain radiates to back and shoulder
Pyrexia if Cholecystitis has developed
What is Cholecystitis?
Inflammation of the gall bladder caused by gallstones
What causes acute Cholecystitis?
A stone getting stuck in the neck of the gall bladder or the cystic duct
This causesa build up of pressure in the gall bladder, which then leads to inflammation of it
Very occasionally it occurs without stones
What causes chronic Cholecystitis?
After many attacks on the gall bladder by gallstones and acute Cholecystitis it becomes damaged.
How does acute Cholecystitis present compared with chronic?
Acute: RUQ pain, radiating to back, jaundice, vomiting, pyrexia, tenderness
Chronic: can be asymptomatic, vague abdominal dysfunction, nausea, fat intolerance
Why does fat intolerance occur in Cholecystitis?
Because fatty food stimulates the gall bladder to contract, causing pain if the gall bladder is damaged.
Investigations of acute Cholecystitis?
Raised white cells
Abnormal liver function tests
- shows gallstones
- distended gall bladder
- thickened wall
Management of acute Cholecystitis?
Nil by mouth to keep gall bladder from contracting
IV fluids, antibiotics
Cholecystectomy within 48 hours
In chronic Cholecystitis do you do a cholecystectomy?
Not unless symptoms indicate to do so
What is the pathophysiology of Cholecystitis?
A blockage in the cystic duct or common bile duct
When the gall bladder contracts the bile can't escape so it stretches the gall bladder and increases pressure
Over time the bile becomes toxic and damages the mucosa causing it to release inflammatory cytokines
Bacteria might start to grow
Describe what pain occurs in Cholestasis?
Mid epigastric pain to start with
As it progresses it localises in the right upper quadrant
This can radiate to the back and shoulder
What is choledocholithiasis?
A stone in the common bile duct
Causes blockage of bile and problems with bladder
Acute pancreatitis as pancreatic enzymes can't get through
What are the clinical features of choledocholithiasis?
The same as Cholecystitis
How do you distinguish choledocholithiasis from Cholecystitis?
Choledocholithiasis: negative Murphy's sign
What is Murphy's sign?
The patient is instructed to inspire
Doctor presses down on the site of the gallbladder
If the patient stops breathing in (as the gallbladder is tender and, in moving downward, comes in contact with the examiner's fingers) and winces with a 'catch' in breath, the test is considered positive.
In order for the test to be considered positive, the same manoeuver must not elicit pain when performed on the left side.
How do you treat choledocholithiasis?
Removal of stone with ERCP or laparoscopy
What is ascending cholangitis?
Infection of the biliary tree
Why is it called 'ascending' cholangitis?
No particular reason, it is also simply called cholangitis
What causes ascending cholangitis?
Choledocholithiasis: stone in common bile duct
Benign biliary stricture as a result of surgery
Clinical features of ascending cholangitis?
Pale stools and dark urine
What is Charcot's triad?
A triad of symptoms that are seen in Ascending Cholangitis
1. right upper quadrant pain,
Investigation of ascending cholangitis?
- Raised white cells due to infection
- cultures of pathogen
- raised serum bilirubin + alkaline phosphatase
- dilated common bile duct
- stones visible
- will show the stone
- can get a bile sample
What pathogens usually cause ascending cholangitis?