Immune Mediated Liver Disease Flashcards

1
Q

Autoimmune diseases of the bile duct

A

Primary Biliary Cholangitis

Primary Sclerosing Cholangitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Autoimmune diseases of the liver

A

Autoimmune Hepatitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the main disease affecting the small microscopic bile ducts?

A

PBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Features of PBC

A
More females 9:1
>40 years
Small bile duct destruction
Autoimmune
Raised ALP and GGT
Detect anti-mitochondrial antibody in blood (M2 subunit)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Symptoms of PBC

A

Jaundice - late
Pruritis
Fatigue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the main liver enzymes involved with biliary disease?

A

ALP

Gamma - GGT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How is PBC diagnosed?

A

AMA + persistent chronic cholestasis and other causes excluded
Biopsy - destruction of small bile ductules + inflammation + sometimes granulomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Treatment of PBC

A
Ursodeoxycholic acid( UDCA) - good outcome for 2/3
So..
Obeticholic Acid 2nd line
Bezafibrate 3rd line
Liver Transplantation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

MOA OF UDCA

A

Ursodeoxycholic acid
Derivate of naturally occurring bile acid
Makes bile flow more freely

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Symptom treatment for PBC

A

Itch = cholestyramine, rifampicin
Fatigue
Bones - osteoporosis in chronic post menopausal women

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Features of PSC

A

M>F
>20s
65% have IBD
Large bile duct sclerosis and loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Symptoms of PSC

A

Jaundice - earlier
Pruritus
Cholangitis- infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Cancer & PSC

A

Increased risk

  • cholangiocarcinoma
  • colorectal cancer
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Treatment for PSC

A
  • none effective
  • UDCA sometimes used
  • ERCP for strictures
  • AB for infections
  • liver transplant for progressive disease is often only option
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

ERCP

A

Endoscopic retrograde cholangiopancreatography

  • for dominant strictures
  • to diagnose cholangiocarcinoma
  • balloon dilatation vs. stenting vs. both
  • risks = infection, pancreatitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is a dominant stricture?

A

Stricture in main bile duct which is causing blockage upstream from that point

17
Q

Features of autoimmune hepatitis

A

M:F 1:3

18
Q

Diagnosis of autoimmune hepatitis

A

High ALT
Autoantibodies = SM, ANA, ALSA, LKM
High IgG
Liver Biopsy

19
Q

Associates of autoimmune hepatitis

A
Autoimmune thyroid
Coeliac
SLE
Vitiligo
Addison's
20
Q

Biopsy appearance of autoimmune hepatitis

A

Blue nuclei lots
Lots of inflammatory cells
Interface hepatitis = between bile duct and liver
Plasma cells and lymphocytes

21
Q

Treatment for autoimmune hepatitis

A

High dose steroids = prednisolone, budesonide
Immunomodulators = azathioprine
Failure of 1st line -> mycophenolate/tacrolimus/rituximab
Liver Transplantation

22
Q

How to assess Tx response in autoimmune hepatitis?

A

ALT
IgG
Liver biopsy