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Year 3 - Met3a > Immune mediated diseases of the liver > Flashcards

Immune mediated diseases of the liver Flashcards

1
Q

What are the autoimmune diseases of the bile duct

A
  • primary biliary cholangitis

- primary sclerosing cholangitis

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2
Q

What are the autoimmune diseases of the liver

A
  • autoimmune hepatits
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3
Q

what are the symptoms of primary biliary cholangitis (PBC)

A
  • Often asymptomatic and diagnosed after incidental finding of raised ALP
  • jaundice
  • sleepiness
  • pruritus
  • lethargy
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4
Q

Who tend to have primary biliary cholangitis (PBC)

A
  • more common in women 9;1 to men

- usually aged over 40

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5
Q

What is primary biliary cholangitis (PBC)

A

this is when interlobular ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis which may lead to fibrosis, cirrhosis and portal hypertension

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6
Q

What markers increase in primary biliary cholangitis (PBC)

A
  • increase in ALP

- increase in AMA (M2) - antibody presence used as diagnosis

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7
Q

What investigations do you use to show the presence of primary biliary cholangitis

A
  • Bloods
  • Ultrasound
  • Fibroscan - see how much scarring is in the liver to check for fibrosis and cirrhosis
  • Biospy
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8
Q

What is the treatment for primary biliary cholangitis (PBC)

A
  • Ursodeoxycholic acid (UDCA) - 1st line
  • Obeticholic acid - for people that dont respond to UDCA
  • bezafibrate
  • liver transplantation

for symtpoms

  • itch - cholestyramine, rifampicin
  • bones - risk of osteoporosis - bisphopshonates
  • diarrhoea - codeïne phosphate e.g. 30mg/8hour
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9
Q

What is primary sclerosing cholangitis (PSC) symptoms

A
  • jaundice
  • pruritus
  • cholangitis
  • can be asymptomatic
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10
Q

Who does primary sclerosing cholangitis (PSC) effect

A
  • more males than females
  • age 20 onwards
  • 65% have IBD usually UC
  • HLA-A1, B8, DR3
  • AIH
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11
Q

What happens in primary sclerosing cholangitis (PSC)

A
  • progressive cholestasis and Large bile duct sclerosis and loss
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12
Q

what cancers are you at risk of developing when you have primary sclerosing cholangitis (PSC)

A
  • cholangiocarcinoma
  • colorectal cancer
  • hepatobillary cancer
  • pancreatic cancer
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13
Q

What is the treatment of primary sclerosing cholangitis (PSC)

A

No effective treatment

  • UDCA - not as effective
  • ERCP - stricture treatment
  • liver transplantation - mainstay for end stage disease
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14
Q

What is the role of ERCP for primary sclerosing cholangitis (PSC)

A
  • used for dominant strictures - stricture in the main bile duct that is causing blockage of bile flow
  • treated with balloon dilatation or stenting or both
  • to diagnose cholangiocarcinoma
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15
Q

How does autoimmune hepatitis effect

A

females more than males 1:3

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16
Q

What is autoimmune hepatitis associated with

A
  • Autoimmune thyoid
  • coeliac
  • SLE
  • vitiligo
  • addisons
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17
Q

How do you diagnose autoimmune hepatitis

A
  • High ALT

autoantibodies

  • smooth muscle
  • ANA
  • LKM
  • ALSA
  • IgG is raised
  • liver biopsy
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18
Q

How do you treat autoimmune hepatitis

A

High dose steroids

  • Prednisolone 30mg/d PO for 1 month; ↓ by 5mg/month to a maintenance dose of 5-10mg/d PO
  • Can sometimes be stopped after 2yrs but relapse occurs in 50-86%

Immunomodulators
- Azathioprine - 50-100mg/d PO – as steroid-sparing agent to maintain remission

failure of first line therapy
- mycophenolate/ tacrolimus/rituximab

  • Liver transplantation
19
Q

How do you assess the treatment response to autoimmune hepatitis

A
  • ALT
  • IgG
  • liver biopsy
20
Q

What is autoimmune hepatitis

A
  • inflammatory liver disease of unknown cause characterised by abnormal T cell function and autoantibodies directed against hepatocytes surface antigens
21
Q

What are the signs of autoimmune hepatitis

A
  • fever
  • malaise
  • urticarial rash
  • polyarthrtiis
  • pleurisy
  • pulmonary infiltration
  • glomerulonephritis
22
Q

What are the two types of autoimmune hepatitis

A
  • type 1

- type 2

23
Q

Describe type 1 autoimmune hepatitis

A
  • seen in 80%
  • typical patient is female and under 40 years old
  • ASMA +ve in 80%
  • ANA +ve in 10%
  • good response to immunosuppression in 80%
  • 25% have cirrhosis at presentation
24
Q

Describe type 2 autoimmune hepatitis

A
  • more common in Europe than USA
  • more often seen in children
  • more commonly progresses to cirrhosis
  • less treatable
  • typically LKM1 +ve
  • ASMA and ANA -ve
25
Q

What do the investigations show in autoimmune hepatitis

A
  • LFT as above
  • Hypergammaglobulinaemia (esp IgG)
  • +ve autoantibodies
  • Anaemia
  • Hypersplenism: ↓WCC, ↓platelets
26
Q

What does a liver biopsy show in autoimmune hepatitis

A
  • mononuclear infiltrate of portal and periportal areas and piecemeal necrosis and fibrosis
  • cirrhosis - has worse prognosis
27
Q

What does an MRCP help exclude in autoimmune hepatitis

A
  • helps exclude PSC if ALP is disproportionately high
28
Q

What is the prognosis of autoimmune hepatitis

A
  • appears not to matter whether symptomatic or asymptomatic at presentation = 10 year survival - 80% for both
  • presence of cirrhosis at presentation reduces 10 year survival from 94% to 62%
29
Q

what is the cause of primary biliary cholangitis (PBC)

A
  • unknown environmental triggers and genetic predisposition leading to loss of immune tolerance to self-mitochondrial proteins
30
Q

What antibody is the main one in primary biliary cholangitis

A
  • antimicrobial antibodies (AMA)
31
Q

What are the risk factors for primary biliary cholangitis

A
  • family history
  • many UTIs
  • smoking
  • past pregnancy
  • other autoimmune conditions
  • Marked use of nail polish/hair dye
  • typical age at presentation - 50 years
32
Q

What are the signs of primary biliary cholangitis

A
  • jaundice
  • skin pigmentation
  • Xanthelasma
  • Xanthomata
  • hepatosplenomegaly
33
Q

What are the complications of primary biliary cholangitis

A
  • Those of cirrhosis - hepatic failure, portal hypertension, HCC
  • osteoporosis
  • malabsorption of fat-soluble vitamins (A, D, E, K) due to cholestasis and decreased bilirubin in the gut lien resulting in osteomalacia and coagulopathy
34
Q

What do bloods show in primary biliary cholangitis

A
  • LFT - raised ALP, raised gamma GT, mildly raised AST and ALT
  • late disease = raised bilirubin, decreased albumin, raised PT/INR
  • AMA M2 subtype positive
  • immunoglobulins are raised
  • TSH and cholesterol are raised or normal
35
Q

What does a biopsy show of primary biliary cholangitis

A
  • not usually needed - unless drug induced cholestasis or hepatic sarcoidosis need excluding
  • look for granulomas around bile ducts and cirrhosis
36
Q

What are the side effects of ursodeoxycholic acid

A

weight gain

37
Q

How do you monitor primary biliary cholangitis

A
  • regular LFT

- Ultrasound and AFP twice a year if cirrhotic

38
Q

What is the prognosis of primary biliary cholangitis

A
  • variable

- Mayo model used that combines age, bilirubin, albumin, PT, oedema, and need for diuretics

39
Q

What investigations are done in primary sclerosig cholangitis

A
  • LFT
  • immunoglobulins
  • autoantibodies
  • ERCP/MRCP - reveals duct anatomy and damage
  • liver biopsy - shows a fibrous, obliterative cholangitis
40
Q

What LFTs show during primary sclerosig cholangitis

A
  • raised ALP then raised bilirubin
41
Q

What immunoglobulins are in primary sclerosig cholangitis

A
  • hypergammaglobulinaemia and raised IgM
42
Q

What autoantibodies are in primary sclerosing cholangitis

A
  • AMA negative, but ANA, SMA and ANCA may be positive
43
Q

What autoantibodies are in primary biliary cholangitis

A
  • AMA M2 positive - anti-mitochondrial antibodies

Year 3 - Met3a (43 decks)

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