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AIP: Jaundice > Autoimmune hepato-biliary causes > Flashcards

Autoimmune hepato-biliary causes Flashcards

1
Q

In which patient group is autoimmune hepatitis more commonly seen?

A

young females

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2
Q

What are 3 recognised associations of autoimmune hepatitis?

A
  1. Autoimmune disorders
  2. Hypergammaglobulinaemia
  3. HLA B8 DR3
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3
Q

What can autoimmune hepatitis be based upon?

A

types of circulating antibodies present

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4
Q

What are the 3 types of autoimmune hepatitis and the associated circulating antibodies?

A
  1. Type I: anti-nuclear antibodies (ANA) and/or anti-smooth muscle antibodies (SMA)
  2. Type II: anti-liver/kidney microsomal type 1 antibodies (LKM1)
  3. Type III: soluble liver-kidney antigen
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5
Q

Which age groups does type I autoimmune hepatitis (ANA and SMA) commonly affect?

A

both adults and children

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6
Q

Which age group does type II autoimmune hepatitis (anti-LKM1) commonly affect?

A

affects children only

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7
Q

Which age group does type III autoimmune hepatitis (liver-kidney antigen) most commonly affect?

A

adults in middle-age

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8
Q

What are 8 clinical features of autoimmune hepatitis?

A
  1. Jaundice, fever (acute hepatitis signs)
  2. Signs of chronic liver disease
  3. Fatigue
  4. Loss of appetitie
  5. Hepatomegaly
  6. Splenomegaly
  7. Abdominal pain
  8. Amenorrhoea (common)
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9
Q

What are 4 key investigations for autoimmune hepatitis and what will they show?

A
  1. Deranged LFTs: ALT and bilirubin raised, normal/mildly raised ALP
  2. ANA/SMA/LKM1 antibodies
  3. IgG predominant hypergammaglobulinaemia
  4. Liver biopsy: inflammation extending beyond limiting plate (piecemeal necrosis), bridging necrosis
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10
Q

Which is the commonest type of autoimmune hepatitis?

A

Type 1

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11
Q

What proportion of patients with autoimmune hepatitis Type I will have raised anti-SMA vs raised ANA?

A

80% anti-SMA, 10% ANA

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12
Q

What are 3 aspects of the management of autoimmune hepatitis?

A
  1. Steroids
  2. Other immunosuppressants e.g. azathioprine
  3. Liver transplantation
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13
Q

In which patient group is primary biliary cholangitis most commonly seen?

A

middle-aged females

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14
Q

What is the gender ratio affected by primary biliary cholangitis?

A

F:M 9:1

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15
Q

What is the pathophysioloy of primary biliary cholangitis?

A

interlobular bile ducts become damaged by a chronic inflammatory process, leads to scarring, causing progressive cholestasis which may eventually progress to cirrhosis

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16
Q

What can primary biliary cholangitis progress to?

A

cirrhosis (previously called primary biliary cirrhosis)

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17
Q

What is the classic presentation of of primary biliary cholangitis?

A

itching in a middle-aged woman

18
Q

What are 4 associations of primary biliary cholangitis?

A
  1. Sjogren’s syndrome (up to 80%)
  2. rheumatoid arthritis
  3. systemic sclerosis
  4. thyroid disease
19
Q

What proportion of patients with primary biliary cholangitis also have Sjogren’s syndrome?

A

80%

20
Q

What are 10 clinical features of primary biliary cholangitis?

A
  1. Extreme fatigue
  2. Itching
  3. Dry skin
  4. Dry eyes
  5. Jaundice
  6. Hyperpigmentation, especially over pressure points
  7. Right upper quadrant pain
  8. Clubbing
  9. Hepatosplenomegaly
  10. Xanthelasmata
21
Q

Which antibody type is highly sensitive and specific for primary biliary cholangitis?

A

anti-mitochrondial antibodies (AMA) M2 subtype

22
Q

What are 3 types of antibodies which may be present in PBC and may aid diagnosis?

A
  1. Anti-mitochondrial antibody (AMA) type M2 (98%)
  2. Smooth muscle antibodies (30%)
  3. Raised serum IgM
23
Q

What are 4 types of investigations key to making a diagnosis of PBC?

A
  1. LFTs - deranged
  2. Antibodies: Positive AMA (anti-mitochondrial antibodies), SMA, IgM
  3. Abdomional ultrasound
  4. Liver biopsy - inflammation and scarring
24
Q

Wat are 4 aspects of the management of primary biliary cirrhosis?

A
  1. First line: ursodeoxycholic acid
  2. Cholestyramine for pruritus
  3. Fat-soluble vitamin supplements
  4. Liver transplantation
25
Q

What is the first line management of PBC and what is its effect?

A

Ursodeoxycholic acid: slows disease progression and imrpvoes symptoms

26
Q

What is an indication for liver transplantation in PBC?

A

bilirubin >100

27
Q

What is a problem with liver transplantation in PBC?

A

recurrence in graft can occur - but not usually a problem

28
Q

What are 3 complications of PBC?

A
  1. Cirrhosis - portal hypertension, ascites, variceal haemorrhage
  2. Osteomalacia and osteoporosis
  3. Significantly increased risk of hepatocellular carcinoma (20x risk)
29
Q

Why does PBC have a poor mortality?

A

risk of hepatocellular carcinoma

30
Q

What is primary sclerosing cholangitis?

A

chronic choelstatic disorder characterised by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts, resulting in multifocal biliary strictures

31
Q

What are 3 key associatiosn of primary sclerosing cholangitis?

A
  1. Ulcerative colitis: 4% of patients with UC have PSC but 80% of patients with PSC have UC
  2. Crohn’s (less commoner than UC)
  3. HIV
32
Q

What proportion of patient’s with PSC have ulcerative colitis?

A

80%

33
Q

What are 8 clinical features of primary sclerosing cholangitis?

A
  1. Can be asymptomatic with abnormal LFTs
  2. Hepatomegaly
  3. Jaundice
  4. Right upper quadrant pain
  5. Fatigue
  6. Weight loss
  7. Fevers
  8. Sweats
34
Q

What will LFTs show in PSC?

A

raised bilirubin and ALP (cholestatic picture)

35
Q

What are 6 investigations to perform to diagnose PSC?

A
  1. LFTs (cholestatic picture)
  2. Positive anti-smooth muscle - SMA
  3. Anti-nuclear antibodies - ANA
  4. Myeloperoxidase antineutrophil cytoplasmic antibody - p-ANCA
  5. MRCP: multiple beaded biliary strictures
  6. ERCP: shows beaded biliary strictures
36
Q

What are 3 types of antibodies which may be positive for primary sclerosing cholangitis?

A
  1. ANA
  2. SMA
  3. p-ANCA
37
Q

What are the standard diagnostic investigations for primary sclerosing cholangitis?

A

ERCP or MRCP

38
Q

What may liver biopsy show in PSC and is it indicated?

A

limited role for it

may should fibrous, obliterative cholangitis often described as ‘onion skin’

39
Q

What are 2 complications of PSC?

A
  1. Cholangiocarcinoma (10%)
  2. Increased risk of colorectal cancer
40
Q

What are 5 aspects of the management of PSC?

A
  1. Avoid alcohol
  2. Pruritis can be managed with cholestyramine
  3. Supplement fat-soluble vitamins (ADEK)
  4. Strictures can be dilated via ERCP
  5. Liver transplantation may be indicated in cases complicated by chronic liver disease and/or hepatobiliary malignancies

AIP: Jaundice (10 decks)

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