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GI > PSC, PBC, cholangiocarcinoma > Flashcards

PSC, PBC, cholangiocarcinoma Flashcards

1
Q

What is PBC

A

Chronic autoimmune granulomatous attack on bile duct INSIDE liver
Leads to
Cholestasis so bile, bilirubin and cholesterol can’t be excreted
Cirrhosis
Portal hypertension

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2
Q

What causes PBC

A

Autoimmune
Genetic
Environment

M rule

  • Middle age
  • IgM
  • Anti mitochondrial Ab
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3
Q

What are the symptoms of PBC

A 
None
Incidental raised ALP
Fatigue
Pruritus = classic due to build up of bile 
Jaundice
Dark urine and pale stool 
GI disturbance / malabsorption as lack of bile to digest
RUQ pain
Bone and joint pain
Dry eyes and mouth due to sicca syndrome 
Hyperpigmentation
HSM
Clubbing
Xanthesomata as cholesterol builds up
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4
Q

Who is at risk of PBC

A 
Women
Smoking
Autoimmune
Pregnancy
UTI
Nail polish
FH
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5
Q

What is associated with PBC

A 
Sjogren
Systemic sclerosis
RA
Thyroid
Coeliac
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6
Q

How do you Dx PBC

A

MRCP
Blood - raised ALP, GGT
AMA + IGM = Dx
Increased bilirubin, INR, decreased platelet
Increased TSH?
USS to exclude extra-hepatic
Liver biopsy / cholangiogram not usually needed but confirms

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7
Q

How do you treat PBC

A

No definite
UDCA - flush out salts / reduce cholesterol
Fat soluble vitamin
Treat dry eyes
Treat pruritus - cholestryalmine as decreases bile
Liver transplant if severe

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8
Q

How do you monitor PBC

A

Regular LFT

USS and AFP 6 monthly

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9
Q

What are the complications of PBC

A 
Cirrhosis - HCC 
Decompensated liver
Vitamin deficiency due to fat malabsorption - ADEK
-Osteomalacia 
-OSteoporosis 
-Coagulopathy 
Hypothyroid
Distal renal tubular acidosis
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10
Q

What is PSC

A

Chronic autoimmune inflammation and scarring of intra and extra hepatic ducts
Cholestasis

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11
Q

What causes PSC

A

Unknown

80% = UC

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12
Q

Who is at risk of PSC

A 
Male 
Young adult 
FH
pANCA
ANA +ve
AMA -ve
Autoimmue hepatitis
HIV
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13
Q

What are the symptoms of PSC

A 
Fatigue
CHolestasis = jaundice / pruritus / pale stool 
Abdo pain RUQ
Fever
Heptaomegaly 
Ascending cholangitis if advanced
Cirrhosis
Hepatic failure
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14
Q

How do you Dx PSC

A

Bloods - LFT / ANCA / IgG
USS - look for other cause e.g. gall stone
MRCP = beast diagnostic
ERCP if going to do invasive Rx - beeded due to stricture
Liver biopsyy / cholangiogram not usually needed

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15
Q

How do you treat PSC

A 
UDCA - not proven
Treat itch - cystalimine
Liver transplant = main stay
Endoscopic treatment of bile duct / stent - ERCP 
Ax if infection
Monitor for complications
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16
Q

When do you transplant

A

Chronic and poor QOL
Carcinoma
Genetic
MELD score

17
Q

What do you give before transplant

A

Immunosuppresion
Steroid
Ax

18
Q

What are the complications of PSC

A 
Cirrhosis
Acute bacterial cholangitis 
Intrahepatic obstruction
Cholangiocarcinoma
Colon cancer - screen 
Fat soluble vitamin deficiencies
19
Q

What is advised in patients with PSC

A

Annual colonoscopy especially if UC

20
Q

What is cholangiocarcinoma

A

Cancer of the bile ducts
90% adenocarcinoma
Extrahepatic 80%

21
Q

What are the symptoms of cholangiocarcinoma

A

Obstructive jaundice - gradual onset
Typically painless jaundice
Persistent colic / abdo pain
Weight loss

Other 
Fever 
Anoreixa
Mass in RUQ
LN (peri-umbilical) + Virchow
AScites 
Pruritus
Non-specific - malaise
Hypercalcaemia
Hypophosphate
22
Q

What tumour markers are elevated

A

Ca-125 - ovarian
Ca19-19 - pancreas (raised in cholangiocarcinoma)
CEA - bowel

23
Q

What are the RF for cholangiocarcinoma

A 
PSC / UC
Age
Liver fluke
Gall bladder calcification
Biliary cysts
Chronic liver 
Carcinogens
24
Q

How do you Dx cholangiocarcinoma

A 
Raised bilirubin and ALP
Coag and INR may be affected
USS = 1st line may show dilatation
EUS
Staging = CT / MRI / MRCP
25
Q

How do you treat cholangiocarcinoma

A

Surgery = only option but only 30% operable

Adjuvant chemo / RT

26
Q

What is palliative Rx for cholangiocarcinoma

A

Surgical bypass
ECRP / PTC to stent
Chemo or RT

27
Q

What are post-op complications

A

Liver failure
Bile leek
Bleeding

28
Q

What is Ddx

A

Stone
Cholecystitis
Benign stricture

29
Q

What is ampulla cancer

A

Cancer of ampulla
Adenocarcinoma
Rx = endoscopic excision or pancreaticoduodectomy

30
Q

What do you do for FAP

A

Remove as will cause cancer

31
Q

What is gallbladder cancer

A

Very rare but very aggressive

32
Q

Cholangiocarcinoma vs head of pancreas

A

Head of pancreas = Cuoreviers law
- Painless jaundice + palpable mass as suggest something compressing biliary tree
Can also be biliary malignancy

Cholangiocaricnoma = gradual onset obstruction

33
Q

How do you Dx

GI (29 decks)

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