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Biliary tract diseases Flashcards

1
Q

What is Primary Biliary Cholangitis?

A

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

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2
Q

What are the intrahepatic bile ducts? Where are they?

A

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

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3
Q

Describe the journey of bile from its production to its storage to its release into the small intestine?

A

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

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4
Q

What is cholestasis?

A

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

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5
Q

Which people are most often affected with PBC?

A

Women between age 40-50

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6
Q

What is the difference between Primary Biliary Cirrhosis and Primary Biliary Cholangitis?

A

They are the same!

Primary Biliary Cholangitis is the new name for it

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7
Q

What causes PBC?

A

Genetic predisposition

But the disease is set off in these people by an environmental trigger

Such as infection or pollution

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8
Q

What are the risk factors of developing PBC?

A 
Family history
Being female
Many UTIs
Smoking
Having other autoimmune disease
Past pregnancy
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9
Q

What are the clinical features of PBC?

A

Often found incidentally via LFT blood tests

Pruritus (itching)
Lethargy
Jaundice
Skin pigmentation
Xanthoma

Hepatosplenomegaly

Signs of liver failure:

  • ascites
  • varices
  • hepatic encephalopathy
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10
Q

What is Xanthoma?

A

Deposition of yellowish cholesterol-rich material that can appear anywhere in the body
These can appear on the skin as yellow blob-like lesions

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11
Q

What 2 types of cholestasis are there?

A

Hepatocellular: the hepatocytes aren’t making bile

Obstructive: something is blocking the flow of bile

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12
Q

What type of cholestasis is PBC?

A

Obstructive

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13
Q

What are the complications of PBC?

A

Cirrhosis complications:

  • Portal hypertension
  • Encephalopathy
  • Liver failure

Osteoporosis

Malabsorption of fat soluble vitamins

Coagulopathy

Hepatocellular carcinoma

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14
Q

Which are the fat soluble vitamins?

A

A, D, E, K

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15
Q

Why does PBC cause malabsorption of fat soluble vitamins?

A

Because bile is essential for the digestion of lipids
So if no bile, no lipid absorption

So fat soluble vitamins can’t be absorbed

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16
Q

Why does PBC cause coagulopathy?

A

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

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17
Q

Investigations of PBC?

A

Blood:

  • raised serum alkaline phosphate
  • in late disease, raised bilirubin + low albumin
  • autoantibodies

USS
- exclude extra-hepatic cholestasis: problems with gall bladder or pancreas

Biopsy:

  • not usually needed, but look for granulomata round bile ducts
  • look for cirrhosis + scarring
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18
Q

Management of PBC?

A

Treat the symptoms: pruritus, diarrhoea, osteoporosis

Give fat soluble vitamin supplements to accommodate malabsorption

Drug treatment: UDCA

Regular monitoring of liver function and USS

Liver transplant

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19
Q

What is UDCA?

A

Ursodeoxycholic acid

It reduces ascites, jaundice and can improve survival and delay the need for transplant

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20
Q

What is the prognosis of PBC?

A

Not good

Once jaundice develops, less than 2 years

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21
Q

What is Primary Sclerosing Cholangitis?

A

Progressive obliteration, inflammation and narrowing of intra + extra-hepatic ducts

This leads to cholestasis

Eventually leading to strictures, cirrhosis and gallstones

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22
Q

What is the difference between PBC and PSC?

A

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

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23
Q

Which one of PBC and PSC is linked with inflammatory bowel disease?

A

PSC

Over 50% have both

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24
Q

Clinical presentation of PSC?

A

Pruritus
Fatigue
Pain + rigors

Cirrhosis
Ascending cholangitis
Signs of liver damage
- Jaundice
- Ascites
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25
What are the risk factors for PSC?
Male gender HLA-A1 Also having IBD, most commonly UC
26
What are the complications of PSC?
Liver failure Complications associated with this: portal hypertension, encephalopathy Cancer: - cholangiocarcinoma - gall bladder adenocarcinoma - liver - colon
27
What is cholangiocarcinoma?
Bile duct cancer
28
Investigations of PSC?
Bloods: - raised alkaline phosphate - raised bilirubin - ANCA (autoantibody) ERCP: - do check for extra + intra-hepatic bile duct involvement Biopsy: - shows cholangitis: fibrous scarred bile ducts
29
What is ANCA?
Anti-neutrophil cytoplasmic antibody An autoantibody, linked with autoimmune disease
30
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
31
Management of PSC?
UDCA drug: may improve liver function and protect against colon cancer Liver transplant Treat symptoms: pruritus Screen regularly for cancers of bile duct, gall bladder, liver, colon
32
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
33
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
34
What are the 2 types of gallstones? Which are more common?
Cholesterol: most common - 80% Pigment
35
Why do cholesterol gallstones form?
In bile with excess cholesterol In gall bladders with reduced motility
36
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: - haemolysis - sickle cell disease - cirrhosis
37
What are the risk factors for developing cholesterol gall stones?
``` Older age Being female Family history Multiparity: given birth to many children Obesity Diabetes High fat diet ```
38
Investigations of gallstones?
Evidence from history Blood: - increased serum alkaline phosphate - increased bilirubin - inflammatory features could indicate cholecystitis
39
What problems can gallstones cause?
Biliary colic Cholecystitis: inflammation of gall bladder Cholangitis: inflammation of bile ducts Pancreatitis
40
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
41
What are the clinical features of gallstones?
Severe right upper quadrant pain Pain radiates to back and shoulder Vomiting Jaundice Pyrexia if Cholecystitis has developed
42
What is Cholecystitis?
Inflammation of the gall bladder caused by gallstones
43
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
44
What causes chronic Cholecystitis?
After many attacks on the gall bladder by gallstones and acute Cholecystitis it becomes damaged.
45
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
46
Why does fat intolerance occur in Cholecystitis?
Because fatty food stimulates the gall bladder to contract, causing pain if the gall bladder is damaged.
47
Investigations of acute Cholecystitis?
Raised white cells Abnormal liver function tests USS: - shows gallstones - distended gall bladder - thickened wall
48
Management of acute Cholecystitis?
Nil by mouth to keep gall bladder from contracting IV fluids, antibiotics Analgesia Cholecystectomy within 48 hours
49
In chronic Cholecystitis do you do a cholecystectomy?
Not unless symptoms indicate to do so
50
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 Causing pain Over time the bile becomes toxic and damages the mucosa causing it to release inflammatory cytokines Bacteria might start to grow
51
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
52
What is choledocholithiasis?
A stone in the common bile duct Causes blockage of bile and problems with bladder ALSO Acute pancreatitis as pancreatic enzymes can't get through
53
What are the clinical features of choledocholithiasis?
The same as Cholecystitis
54
How do you distinguish choledocholithiasis from Cholecystitis?
Choledocholithiasis: negative Murphy's sign Cholecystitis: positive
55
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.
56
How do you treat choledocholithiasis?
Removal of stone with ERCP or laparoscopy
57
What is ascending cholangitis?
Infection of the biliary tree
58
Why is it called 'ascending' cholangitis?
No particular reason, it is also simply called cholangitis
59
What causes ascending cholangitis?
Choledocholithiasis: stone in common bile duct Benign biliary stricture as a result of surgery Chronic pancreatitis
60
Clinical features of ascending cholangitis?
``` Fever Jaundice RUQ pain Rigors Skin itching Pale stools and dark urine ``` Shock
61
What is Charcot's triad?
A triad of symptoms that are seen in Ascending Cholangitis 1. right upper quadrant pain, 2. jaundice 3. fever
62
Investigation of ascending cholangitis?
Bloods: - Raised white cells due to infection - cultures of pathogen - raised serum bilirubin + alkaline phosphatase USS: - dilated common bile duct - stones visible ERCP: - will show the stone - can get a bile sample
63
What pathogens usually cause ascending cholangitis?
E. coli | Enterococcus faecalis
64
Management of ascending cholangitis?
Resuscitate if in shock Analgesia Antibiotics Relief of obstruction: ERCP

Liver (10 decks)

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