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GI: liver, biliary tree and pancreas > Autoimmune Liver Disease: chronic liver disease > Flashcards

Autoimmune Liver Disease: chronic liver disease Flashcards

1
Q

what are 5 examples of autoimmune liver diseases ?

A
  1. Primary Biliary Cholangitis (Cirrhosis)
  2. Autoimmune Hepatitis
  3. Primary Sclerosing Cholangitis- Diseases with autoimmune features
  4. Alcohol related liver disease
  5. Drug Reactions
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2
Q

what is the pathology of primary bililary cholangitis/cirrhosis ?

A

Interlobular bile ducts are damaged by chronic antoimmune granulomatous inflammation causing cholestasis (destrction of bile ducts) which may lead to fibrosis, cirrhosis and portal hypertension.

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3
Q

aetiology of PBC: __ ___ ___ (AMA) are found in almost all patients and the mitochondiral protein antigen M2 is involved. Autoantigens M2-E2 E3 subunits of ___ ____ ____ - E2 in the inner leaflet of the mitochondrial membrane triggers AMA

A

anti-mitochondrial antibodies (AMA) are found in almost all patients and the mitochondiral protein antigen M2 is involved. Autoantigens M2-E2 E3 subunits of pyruvate dehydrogenase complex - E2 in the inner leaflet of the mitochondrial membrane triggers AMA

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4
Q

PBC is T-cell mediated, CD__ cells become reactive to __ target. There is a loss of tolerance for the __ mitochondrial protein.

A

T-cell mediated, CD4 cells become reactive to M2 target. There is a loss of tolerance for the M2 mitochondrial protein.

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5
Q

PBC is more common in men? true /false

A

false

f:m 10: 1

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6
Q

PBC : what are the symptoms ?

A
  • usually assymptomatic and the diagnosis is incidental

- fatigue

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7
Q

what are the signs of PBC?

A
  • itch without rash
  • xanthelomas
  • jaundice
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8
Q

risk of PBC is increased if: 6

A

Family history, may UTIs, smoking, past pregnancy, other autoimmune, increased use of hair dye and nail polish

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9
Q

diagnosis of PBC is made by 2 of these 3

A
  • positive AMA
  • cholestatic LFTs
  • liver biopsy
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10
Q

what would you be looking for on a liver biopsy for PBC?

A

granulomas around bile ducts ± cirrhosis

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11
Q

what is the treatment for PBC?

A

urseo deoxycholic acid- this reduces inflammation and reduces secondary bile damage

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12
Q

what are the outcomes of PBC?

A

many will not develop symptoms

still a common cause of liver transplant

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13
Q

autoimmune hepatitis pathognesis: This is an inflammatory liver disease of unknown cause. It is thought that an environmental agent (maybe a ___,___, ___) causes a sequence of _ cell mediated events against liver _____, producing a progressive ________ process which results in ___ and ____.

A

This is an inflammatory liver disease of unknown cause . It is thought that an environmental agent (maybe a virus, toxins, drugs) causes a sequence of T cell mediated events against liver antigens, producing a progressive necroinflammatory process which results in fibrosis and cirrhosis.

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14
Q

what drugs could be implicated in autoimmune hepatitis? 6

A

Oxyphenisatin; Methyldopa; Nitrofurantoin; Diclofenac; Minocycline; statins

Most drug reactions go away after the drug is stopped, that is not the case with this one

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15
Q

autoimmune hepatitis affects more women that men. true/false?

A

true

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16
Q

in autoimmune hepatitis (AH) if it is untrated __% will die within 6 months and __% develop cirrhosis

A

40

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17
Q

how many types of AH are there?

A

two

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18
Q

which group of people get type 1 AH?>

A

adults

19
Q

what are the common associations of AH type 1? 3

A

associated with extrahepatic manifestations: autoimmune thyroiditis, graves disease, chronic UC

20
Q

what antibodies and immunoglobulins may be raised in AH type 1?

A
  • ANA positive , ASMA 1 in 40, AMA and pANCA may be positive
21
Q

which Ig are elevated in AH type 1?

A

IgG

22
Q

what are the less common associations of AH type 1? 5

A

RA, perinicious anaemia, systemic sclerosis, ITP, SLE

23
Q

in type 1 AH 40% present with acute onset symptoms similar to ____ ____ or acute ___ ____

A

acute onset symptoms similar to toxic hepatitis or acute viral hepatitis

24
Q

what are the symptoms of AH type 1 ? 6

A

malaise fatigue, lethargy, nausea, abdo pain, anorexia

25
Q

what are the signs of AH type 1? 7

A

Hepatomegaly; jaundice; stigmata of chronic liver disease; splenomegaly; elevated AST and ALT; elevated PT

26
Q

which group of people get type 2 AH?

A

children and young adults

27
Q

which antibodies are associated with AH type 1?

A

LKM-1 antibody and AMA may be present

28
Q

diagnosis of AH is made by: elevated ___, __ ( enzymes which indicate liver damage) and Ig___. rule out other causes. presence of autoimmune ______. Finally liver ____ is the gold standard

A

ALT and AST

IgG

antibodies

biopsy

29
Q

what is the histological hallmark of AH?

A

interface hepatitis

  • marked piecemeal necrosis and lobular involvement
  • numerous plasma cells as well
30
Q

what are the treatment options for AH?

A
  1. corticosteroid treatment - prednisolone

2. prednisolone with azathioprine (steroid sparing immunosuppressant)

31
Q

what are the guidelines for use of prednisolone in AH treatent?

A

start at 30mg daily and taper down to 15mg at week 4, then maintain on 10mg daily until therapy endpoint

32
Q

__% patients with AH will develop eosophageal varices within 2 years

A

54

33
Q

13-20 % of patients with AH will have spontaneous ____

A

resolution

34
Q

what is primary sclerosing cholangitis (PSC) ?

A

This is an autoimmune destructive disease of large and medium sized bile ducts.

35
Q

what is the difference between PBC and PSC?
PBC - ______ invasion of ______ bile duct due to anti-mitochondrial antibody so increased Ig__. And you can get secondary _____ and ____.
PSC - _____ ducts, _____ raised , bile lakes and _____ formation

A

PBC - lymphocyte invasion of intrahepatic bile duct due to anti-mitochondrial antibody so increased IgM. And you can get secondary hypercholesterolemia and xanthelasma.
PSC - Extrahepatic ducts, ANCA, bile lakes and stricture formation

36
Q

PSC more common in males, true/false?

A

true

37
Q

40% of people with PSC also have ____

A

colitis

38
Q

> 80% of northern european patients also have ___ which often presents before PSC

A

IBD

39
Q

risk of ___ ____ is increased in PSC

A

colorectal malignancy

40
Q

what are the symptoms of PSC?

A

pruritus ± fatigue

41
Q

how is it diagnosed? 2

A
  • liver function tests and liver biopsy
42
Q

what things are shown on liver function tests for PSC? 3

A
  • increased alk phos, then increased bilirubin and hypergammaglobulinaemia
43
Q

what is shown on a liver biopsy of PSC?

A

shows a fibrous, obliterative cholangitis

44
Q

what is the management for PSC 2

A
  1. maintain bile flow

2. monitor for cholangiocarcinoma and colorectal cancer

GI: liver, biliary tree and pancreas (20 decks)

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