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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

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2
Q

What is the causative mechanism of auto-immune hepatitis?

A

Auto-antibodies against liver compounds

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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

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4
Q

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

A

Cirrhosis & liver failure

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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

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6
Q

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

A

25-34%

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7
Q

What are the biomarkers used in auto-immune hepatitis?

A
  1. Aminotransferases (ALAT/ASAT)
  2. Hyperglobulinaemia
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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

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9
Q

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

A

30%

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10
Q

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

A

20%

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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
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12
Q

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

A

40%

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13
Q

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

A

False; the presentation is very aspecific

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14
Q

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

A

Females = 75%

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15
Q

What is the therapy for auto-immune hepatitis?

A

Immunosuppressives (prednisone, azathioprine)

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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

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17
Q

How many subtypes of auto-immune hepatitis are there?

A

3

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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

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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)
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20
Q

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

A

F:M = 4:1

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21
Q

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

A

Good response to therapy

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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)
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23
Q

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

A

F:M = 9:1

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24
Q

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

A

Southern Europe

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25
Q

In which age group is auto-immune hepatitis type 2 most common?

A

Predominantly in children -> median age = 10

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26
Q

How is the therapy response of auto-immune hepatitis type 2?

A

Poor response to treatment

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27
Q

What are the characteristic antibodies of auto-immune hepatitis type 3? (2)

A
  1. Anti-SLA (=soluble liver antigen)
  2. Anti-LP (=liver-pancreas)
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28
Q

How is the therapy resposne of auto-immune hepatitis type 3?

A

Good therapy response

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29
Q

What is auto-immune hepatitis overlap syndrome?

A

An overlap between auto-immune hepatitis and primary sclerosing cholangitis

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30
Q

Which factors can predispose to developing auto-immune hepatitis? (4)

A
  1. HLA-D variants
  2. Non-MHC related polymorphisms
  3. Hormonal status
  4. Impaired negative selection of autoreactive T-cells
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31
Q

Which non-MHC related polymorphisms can contribute to the development of auto-immune hepatitis? (3)

A
  1. CTLA-4
  2. TNF-genes
  3. Vitamin D receptor
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32
Q

What are the possible initiating events of auto-immune hepatitis?

A
  1. Viral infection
  2. Drug/toxic-induced liver damage
  3. Molecular mimicry
  4. Neoantigen formation
  5. Bystander activatoin
  6. Hepatic trapping of activated cells
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33
Q

Which evidence supports the theory that auto-immune hepatitis may have a viral trigger?

A

Chronic HBV/HCV patients often also develop ANAs and anti-SMA antibodies

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34
Q

What is often the targer antigen of auto-immune hepatitis?

A

CYP2D6

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35
Q

What are the killing mechanisms in auto-immune hepatitis? (2)

A
  1. FAS(L)-mediated killing by T-cells
  2. ADCC
36
Q

How long does immunosuppression for auto-immune hepatitis typically have to be continued?

37
Q

What alternatives for immunosuppression to treat auto-immune hepatitis are currently being investigated? (4)

A
  1. Adoptive Treg transfer
  2. Modulation of cytokine milieu
  3. Interference with aberrant AhR-signalling
  4. Boost purigenic signalling
38
Q

Which kind of bile ducts are affected by primary biliary cholangitis?

A

Small intrahepatic bile ducts

39
Q

What are the results of bile duct damage in primary biliary cholangitis? (3)

A
  1. Cholestasis
  2. Inflammation
  3. Cirrhosis (long-term)
40
Q

Which antibody is frequently present in primary biliary cholangitis? In how many % of cases?

A

Anti-mitochondrial antibody (AMA) positive in 90% of cases

41
Q

What are the symptoms of primary biliary cholangitis? (7)

A
  1. Fatigue
  2. Pruritus
  3. Jaundice
  4. Abdominal pain
  5. Arthralgia
  6. Dry eyes & mucosa
  7. Osteopenia
42
Q

Primary biliary cholangitis occurs more frequently in [females/males]

A

Females (ratio = 9:1)

43
Q

What is the F:M ratio of primary biliary cholangitis?

44
Q

What is the prevalence of primary biliary cholangitis?

A

20-40/100.000

45
Q

What is the treatment of primary biliary cholangitis?

A

Ursodeoxycholic acid

46
Q

What are the functions of ursodeoxycholic acid in the treatment of primary biliary cholangitis? (3)

A
  1. Replaces toxic bile
  2. Increases bile secretion
  3. Inhibits immune system
47
Q

Against which epitope are the anti-mitochondrial antibodies (AMA) in primary biliary cholangitis aimed?

48
Q

What is the isotype of the anti-mitochondrial antibodies (AMA) in primary biliary cholangitis?

49
Q

Which infections share close homology to PDC-E2 and could possibly be responsible for molecular mimicry leading to primary biliary cholangitis? (2)

A
  1. Mycoplasma
  2. E. coli
50
Q

How are bile ducts protected from the toxic effects of bile acids?

A

The presence of bicarbonate umbrella, maintained by the AE2 pump in the bile duct epithelium

51
Q

What happens to bile acids when they react with bicarbonate?

A

They become less volatile

52
Q

What happens to the amount of AE2 pumps in the bile duct epithelium in primary biliary cholangitis? What does this result in?

A

Downregulation -> epithelium is less protected against effects of bile ducts -> increased apoptosis

53
Q

How has the downregulation of AE2 been hypothesized to influence the pathogenesis of primary biliary cholangitis?

A

Downregulation of AE2 leads to increased apoptosis = release of antigens -> could be the initial step to generating auto-antibodies

54
Q

Which kinds of bile ducts are attacked in primary sclerosing cholangitis?

A

Larger intrahepatic & extrahepatic bile ducts

55
Q

What is the main cause of cholestasis in primary sclerosing cholangitis?

A

Structures of the bile ducts

56
Q

With which disease is primary sclerosing cholangitis frequently associated? How many PSC-patients also have this disease?

A

IBD -> 70%

57
Q

What is the prevalence of primary sclerosing cholangitis?

A

0-16,2/100.000

58
Q

Primary sclerosing cholangitis is most common in [females/males]

A

Males (70%)

59
Q

What is the peak incidence age of primary sclerosing cholangitis?

60
Q

How many % of primary sclerosing cholangitis patients are asymptomatic?

61
Q

What are the symptoms of primary sclerosing cholangitis? (5)

A
  1. Jaundice
  2. Fatigue
  3. Pruritus
  4. Weight loss
  5. Cholangitis
62
Q

What are the complications of primary sclerosing cholangitis? (3)

A
  1. Ascites
  2. Hypersplenism
  3. Variceal bleeds
63
Q

What are the diagnostic markers of primary sclerosing cholangitis?

A
  1. Elevated alkaline phosphatase (AP)
  2. Elevated γ-GT (=liver enzyme)
  3. Elevated transaminases (ALAT/ASAT)
64
Q

What is the standard treatment of primary sclerosing cholangitis?

A

No standard treatment

65
Q

Which treatment strategies are sometimes explored in primary sclerosing cholangitis? (2)

A
  1. Immunosuppressants
  2. Anti-TNFH
66
Q

How many % of primary sclerosing cholangitis patients require LTx?

67
Q

What is the target auto-antigen of primary sclerosing cholangitis?

68
Q

How many % of primary biliary cholangitis patients also has auto-immune hepatitis?

69
Q

What is the mean age of incidence of primary biliary cholangitis?

70
Q

What is the characteristic histological pattern of auto-immune hepatitis?

A

Chronic disease: changes in the portal area & interface hepatis
1. Portal & periportal fibrosis
2. Portal & periportal inflammation (presence of lymphocytes & plasma cells)

71
Q

What is the histological presentation of fulminant auto-immune hepatitis?

A

Lobular inflammation & damage, with multinucleated hepatocytes

72
Q

What is the role of liver biopsy in the diagnostics of auto-immune hepatitis?

A

Biopsy can give more certainty when diagnosis based on other markers is unclear

73
Q

What ist he role of liver biopsy in primary biliary cholangitis?

A
  1. Diagnosis
  2. Staging information (progression & response to therapy)
74
Q

True or false: biopsy is a routine part of the diagnostics of primary biliary cholangitis

A

False; biopsy is not routinely performed

75
Q

What is the characteristic histological presentation of primary biliary cholangitis?

A

Bile duct injury with non-suppurative cholangitis

76
Q

How is the distribution of damaged bile ducts in primary biliary cholangitis?

A

Heterogeneous -> different parts of the liver may or may not be affected

77
Q

Which two types of bile duct injury can be seen in primary biliary cholangitis?

A
  1. Lymphocytic cholangitis (lymphocytes in bile duct epithelium)
  2. Granulomatous cholangitis (=granuloma surrounding the bile duct)
78
Q

What does persistent bile duct injury lead to in the short- and long-term?

A

Short-term: ductular reaction
Long-term: duct loss & ductopenia

79
Q

What is a ductular reaction?

A

Formation of new bile ducts to transfer bile out of the parenchyma

80
Q

What happens when bile ducts begin to leak in primary biliary cholangitis?

A

Neutrophilic responses

81
Q

How can original bile ducts still be recognized in case of duct loss?

A

Aggregates of inflammatory cells and/or fibrous scarring

82
Q

Which marker is characteristic of scar tissue after bile duct loss?

83
Q

How does portal inflammation present itself in primary biliary cholangitis? (5_

A
  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
Q

Which changes to the liver parenchyma can occur in primary biliary cholangitis?

A
  1. Cholate stasis
  2. Copper deposition
  3. Bile plugs
  4. Fibrosis
85
Q

What is the histological feature of cholate stasis?

A

Swollen hepatocytes

I&I WC2 (18 decks)

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