Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

I&I WC2 > Hepatology - Auto-immunity > Flashcards

Hepatology - Auto-immunity Flashcards

1
Q

Which isotype of antibody is increased in auto-immune hepatitis?

A

IgG

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

What is the causative mechanism of auto-immune hepatitis?

A

Auto-antibodies against liver compounds

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

True or false: auto-immune hepatitis often has an acute presentation

A

False: it is frequently not recognized, allowing for cirrhosis & liver failure to occur

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

What can be the consequence of delayed diagnosis of auto-immune hepatitis?

A

Cirrhosis & liver failure

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

True or false: auto-immune disease of the liver is relatively common

A

False; the liver is a very tolerogenic organ and thus does not have many auto-immune diseases

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

How many % of auto-immune hepatitis patients is completely asymptomatic?

A

25-34%

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

What are the biomarkers used in auto-immune hepatitis?

A
  1. Aminotransferases (ALAT/ASAT)
  2. Hyperglobulinaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the symptoms of auto-immune hepatitis? (4) What is the difficulty with these symptoms?

A
  1. Fatigue
  2. Anorexia
  3. Weight loss
  4. Nausea

All aspecific symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How many % of auto-immune hepatitis patients have cirrhosis at presentation?

A

30%

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

How many % of auto-immune hepatitis patients has another auto-immune disease?

A

20%

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

What are the most common auto-immune diseases as co-morbidities with auto-immune hepatitis? (4)

A
  1. Thyroiditis
  2. Primary biliariy cholangitis
  3. Primary sclerosing cholangitis
  4. Diabetes mellitus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How many % of auto-immune hepatitis patients has a family history of auto-immune disease?

A

40%

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

True of false: auto-immune hepatitis has a characteristic histopathological presentation

A

False; the presentation is very aspecific

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

Auto-immune hepatitis predominantly occurs in [females/males]

A

Females = 75%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the therapy for auto-immune hepatitis?

A

Immunosuppressives (prednisone, azathioprine)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

True of false: auto-immune hepatitis always has the same causative antibodies

A

False; there are 3 subtypes of AIH, each with characteristic antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How many subtypes of auto-immune hepatitis are there?

A

3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

How many % of auto-immune hepatitis is subtype 1, 2 and 3?

A

Type 1 = 80%
Type 2 = 20%
Type 3 = very rare

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What are the characteristic auto-antibodies of auto-immune hepatitis type 1? (2)

A
  1. ANA (=anti-nuclear antibody)
  2. Anti-SMA (=smooth muscle antibody)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the F:M ratio of auto-immune hepatitis type 1?

A

F:M = 4:1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

How is the therapy response of auto-immune hepatitis type 1?

A

Good response to therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are the characteristic antibodies of auto-immune hepatitis type 2? (2)

A
  1. Anti-LKM1 (=liver kidney microsomal)
  2. Anti-LC-1 (=liver cytosol)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the F:M ratio of auto-immune hepatitis type 2?

A

F:M = 9:1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

In which geographical area is auto-immune hepatitis type 2 most common?

A

Southern Europe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
In which age group is auto-immune hepatitis type 2 most common?
Predominantly in children -> median age = 10
26
How is the therapy response of auto-immune hepatitis type 2?
Poor response to treatment
27
What are the characteristic antibodies of auto-immune hepatitis type 3? (2)
1. Anti-SLA (=soluble liver antigen) 2. Anti-LP (=liver-pancreas)
28
How is the therapy resposne of auto-immune hepatitis type 3?
Good therapy response
29
What is auto-immune hepatitis overlap syndrome?
An overlap between auto-immune hepatitis and primary sclerosing cholangitis
30
Which factors can predispose to developing auto-immune hepatitis? (4)
1. HLA-D variants 2. Non-MHC related polymorphisms 3. Hormonal status 4. Impaired negative selection of autoreactive T-cells
31
Which non-MHC related polymorphisms can contribute to the development of auto-immune hepatitis? (3)
1. CTLA-4 2. TNF-genes 3. Vitamin D receptor
32
What are the possible initiating events of auto-immune hepatitis?
1. Viral infection 2. Drug/toxic-induced liver damage 3. Molecular mimicry 4. Neoantigen formation 5. Bystander activatoin 6. Hepatic trapping of activated cells
33
Which evidence supports the theory that auto-immune hepatitis may have a viral trigger?
Chronic HBV/HCV patients often also develop ANAs and anti-SMA antibodies
34
What is often the targer antigen of auto-immune hepatitis?
CYP2D6
35
What are the killing mechanisms in auto-immune hepatitis? (2)
1. FAS(L)-mediated killing by T-cells 2. ADCC
36
How long does immunosuppression for auto-immune hepatitis typically have to be continued?
Life-long
37
What alternatives for immunosuppression to treat auto-immune hepatitis are currently being investigated? (4)
1. Adoptive Treg transfer 2. Modulation of cytokine milieu 3. Interference with aberrant AhR-signalling 4. Boost purigenic signalling
38
Which kind of bile ducts are affected by primary biliary cholangitis?
Small intrahepatic bile ducts
39
What are the results of bile duct damage in primary biliary cholangitis? (3)
1. Cholestasis 2. Inflammation 3. Cirrhosis (long-term)
40
Which antibody is frequently present in primary biliary cholangitis? In how many % of cases?
Anti-mitochondrial antibody (AMA) positive in 90% of cases
41
What are the symptoms of primary biliary cholangitis? (7)
1. Fatigue 2. Pruritus 3. Jaundice 4. Abdominal pain 5. Arthralgia 6. Dry eyes & mucosa 7. Osteopenia
42
Primary biliary cholangitis occurs more frequently in [females/males]
Females (ratio = 9:1)
43
What is the F:M ratio of primary biliary cholangitis?
F:M = 9:1
44
What is the prevalence of primary biliary cholangitis?
20-40/100.000
45
What is the treatment of primary biliary cholangitis?
Ursodeoxycholic acid
46
What are the functions of ursodeoxycholic acid in the treatment of primary biliary cholangitis? (3)
1. Replaces toxic bile 2. Increases bile secretion 3. Inhibits immune system
47
Against which epitope are the anti-mitochondrial antibodies (AMA) in primary biliary cholangitis aimed?
PDC-E2
48
What is the isotype of the anti-mitochondrial antibodies (AMA) in primary biliary cholangitis?
IgM
49
Which infections share close homology to PDC-E2 and could possibly be responsible for molecular mimicry leading to primary biliary cholangitis? (2)
1. Mycoplasma 2. E. coli
50
How are bile ducts protected from the toxic effects of bile acids?
The presence of bicarbonate umbrella, maintained by the AE2 pump in the bile duct epithelium
51
What happens to bile acids when they react with bicarbonate?
They become less volatile
52
What happens to the amount of AE2 pumps in the bile duct epithelium in primary biliary cholangitis? What does this result in?
Downregulation -> epithelium is less protected against effects of bile ducts -> increased apoptosis
53
How has the downregulation of AE2 been hypothesized to influence the pathogenesis of primary biliary cholangitis?
Downregulation of AE2 leads to increased apoptosis = release of antigens -> could be the initial step to generating auto-antibodies
54
Which kinds of bile ducts are attacked in primary sclerosing cholangitis?
Larger intrahepatic & extrahepatic bile ducts
55
What is the main cause of cholestasis in primary sclerosing cholangitis?
Structures of the bile ducts
56
With which disease is primary sclerosing cholangitis frequently associated? How many PSC-patients also have this disease?
IBD -> 70%
57
What is the prevalence of primary sclerosing cholangitis?
0-16,2/100.000
58
Primary sclerosing cholangitis is most common in [females/males]
Males (70%)
59
What is the peak incidence age of primary sclerosing cholangitis?
40
60
How many % of primary sclerosing cholangitis patients are asymptomatic?
15-55%
61
What are the symptoms of primary sclerosing cholangitis? (5)
1. Jaundice 2. Fatigue 3. Pruritus 4. Weight loss 5. Cholangitis
62
What are the complications of primary sclerosing cholangitis? (3)
1. Ascites 2. Hypersplenism 3. Variceal bleeds
63
What are the diagnostic markers of primary sclerosing cholangitis?
1. Elevated alkaline phosphatase (AP) 2. Elevated γ-GT (=liver enzyme) 3. Elevated transaminases (ALAT/ASAT)
64
What is the standard treatment of primary sclerosing cholangitis?
No standard treatment
65
Which treatment strategies are sometimes explored in primary sclerosing cholangitis? (2)
1. Immunosuppressants 2. Anti-TNFH
66
How many % of primary sclerosing cholangitis patients require LTx?
>50%
67
What is the target auto-antigen of primary sclerosing cholangitis?
Unknown
68
How many % of primary biliary cholangitis patients also has auto-immune hepatitis?
5-20%
69
What is the mean age of incidence of primary biliary cholangitis?
55
70
What is the characteristic histological pattern of auto-immune hepatitis?
Chronic disease: changes in the portal area & interface hepatis 1. Portal & periportal fibrosis 2. Portal & periportal inflammation (presence of lymphocytes & plasma cells)
71
What is the histological presentation of fulminant auto-immune hepatitis?
Lobular inflammation & damage, with multinucleated hepatocytes
72
What is the role of liver biopsy in the diagnostics of auto-immune hepatitis?
Biopsy can give more certainty when diagnosis based on other markers is unclear
73
What ist he role of liver biopsy in primary biliary cholangitis?
1. Diagnosis 2. Staging information (progression & response to therapy)
74
True or false: biopsy is a routine part of the diagnostics of primary biliary cholangitis
False; biopsy is not routinely performed
75
What is the characteristic histological presentation of primary biliary cholangitis?
Bile duct injury with non-suppurative cholangitis
76
How is the distribution of damaged bile ducts in primary biliary cholangitis?
Heterogeneous -> different parts of the liver may or may not be affected
77
Which two types of bile duct injury can be seen in primary biliary cholangitis?
1. Lymphocytic cholangitis (lymphocytes in bile duct epithelium) 2. Granulomatous cholangitis (=granuloma surrounding the bile duct)
78
What does persistent bile duct injury lead to in the short- and long-term?
Short-term: ductular reaction Long-term: duct loss & ductopenia
79
What is a ductular reaction?
Formation of new bile ducts to transfer bile out of the parenchyma
80
What happens when bile ducts begin to leak in primary biliary cholangitis?
Neutrophilic responses
81
How can original bile ducts still be recognized in case of duct loss?
Aggregates of inflammatory cells and/or fibrous scarring
82
Which marker is characteristic of scar tissue after bile duct loss?
Keratin 7
83
How does portal inflammation present itself in primary biliary cholangitis? (5_
1. Lymphocytes, eosinophils & plasma cells 2. Formation of lymphoid follicles 3. Non-necrotizing granuloma formation 4. Bile duct loss 5. Neutrophil influx (in case of bile duct seepage)
84
Which changes to the liver parenchyma can occur in primary biliary cholangitis?
1. Cholate stasis 2. Copper deposition 3. Bile plugs 4. Fibrosis
85
What is the histological feature of cholate stasis?
Swollen hepatocytes

I&I WC2 (25 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions