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Y3M - Gastro > Autoimmune Liver Disease > Flashcards

Autoimmune Liver Disease Flashcards

1
Q

Give examples of autoimmune liver disease.

A

Primary biliary cholangitis (PBC)

Primary sclerosing cholangitis (PSC)

Autoimmune hepatitis

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2
Q

What is PBC?

A

Interlobular bile ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis which may lead to fibrosis, cirrhosis and portal HTN.

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3
Q

Cause of PBC.

A

Unknown environmental triggers + genetic predisposition leading to loss of immune tolerance to self-mitochondrial proteins.

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4
Q

What is the hallmark antibody for PBC?

A

Antimitochondrial antibodies (AMA)

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5
Q

Prevalence of PBC.

A

<4/100000

Female 9:1 Male

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6
Q

Risk factors of PBC.

A

+ve FH

Many UTIs

Smoking

Past pregnancy

Other autoimmune conditions

Use of nail polish and hair dye.

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7
Q

Typical age of presentation of PBC.

A

50 years old

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8
Q

Clinical features of PBC.

A

Usually asymptomatic and diagnosed after incidental finding of increased ALP.

Lethargy, sleepiness and pruritus may precede jaundice by years.

Jaundice

Skin pigmentation

Xanthelasma

Xanthoma

Hepatosplenomegaly

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9
Q

Complications of PBC.

A

Cirrhotic complications

Osteoporosis

Malabsorption of fat-soluble vitamins (A, D, E, K) due to the cholestasis

Osteomalacia

Coagulopathy

HCC

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10
Q

Blood test findings in PBC.

A

Increased ALP and gamma GT

Mildly increased or normal AST and ALT

Increase in bilirubin and decrease in albumin.

Increase in prothrombin time.

AMA +ve

Other autoantibodies might be present.

Immunoglobulins are increased and especially IgM

TSH and cholesterol might be increased or normal

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11
Q

Why might you do an ultrasound in PBC?

A

To exclude extrahepatic cholestasis.

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12
Q

Biopsy findings in PBC.

A

This is usually not needed unless drug-induced cholestasis or hepatic sarcoidosis needs to be excluded.

Granulomas around the bile ducts and cirrhosis can be present.

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13
Q

Treatment of PBC.

A

Treat the symptoms;
Pruritus - try colestyramine 4-8g/24h PO, naltrexone or rifampicin
Diarrhoea - codeine phosphate 30mg/8h PO
Osteoporosis management

Give fat-soluble vitamin prophylaxis of A, D, E and K.

Consider high-dose ursodeoxycholic acid which can improve survival and delay transplantation.

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14
Q

Monitoring of PBC.

A

Regular LFTs

Ultraound and AFP twice yearly if cirrhotic

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15
Q

Indications for liver transplantation in PBC.

A

End-stage disease or intractable pruritus.

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16
Q

Prognosis of PBC.

A

Highly variable

Mayo survival model is a validated predictor of survival that combines age, bilirubin, albumin, PT time, oedema and need for diuretics.

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17
Q

What is primary sclerosing cholangitis? (PSC)

A

Progressive cholestasis with bile duct inflammation and strictures.

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18
Q

Clinical features of PSC.

A

Jaundice

Chronic right upper quadrant pain

Pruritus

Fatigue

Hepatomegaly

If it is advanced you might see ascending cholangitis, cirrhosis and hepatic failure.

19
Q

Associations of PSC.

A

Male sex

HLA-A1, B8, DR3

Autoimmune hepatitis

80% of northern europena patients also have IBD (usually UC)

20
Q

Cancer risks in PSC.

A

Bileduct cancer

Gallbladder cancer

Liver cancer

Colon cancer

21
Q

How are cancers in PSC monitored?

A

Yearly colonscopy and ultrasound

Consider doing a cholecystectomy for gallblader polyps.

22
Q

Test findings in PSC.

A

Increased ALP and bilirubin

Hypergammaglobulinaemia

Increased IgM

AMA -ve

Can be ANA, SMA or ANCA positive.

23
Q

Gold standard investigation for diagnosis of PSC

A

MRCP

Will show bile duct lesions and strictures

24
Q

Findings on ERCP or MRCP in PSC.

A

Abnormal duct anatomy and damage.

It can show strictures and a characteristc beaded appearance.

MRCP might show intrahepatic ducts with multifocal strictures.

These strictures might be hard to distinguish from cholangiocarcinoma.

25
Q

Treatment of PSC.

A

Ursodeoxyholic acid can improve LFTs but has not shown evidence of survival benefit. High doses may also be harmful, which separates this from PBC.

ERCP to dilate and stent any strictures

Colestryamine for pruritus or naltrexone or rifampicin.

Monitor for any complications

Antibiotics for bacterial cholangitis.

Liver transplant (curative)

26
Q

Indications for liver transplant in PSC.

A

It is the mainstay treatment for end-stage disease and will eventually be required.

27
Q

Findings on liver biopsy of PSC.

A

Fibrous obliterative cholangitis

28
Q

What is autoimmune hepatitis (AIH)?

A

An inflammatory liver disease of unknown cause.

It is characterised by abnormal T-cell function and autoantibodies directed against hepatocyte surface antigens.

29
Q

How is classification of AIH done?

A

By autoantibodies

30
Q

Types of autoimmune hepatitis.

A

Type I

Type II

31
Q

What is type I AIH?

A

Seen in 80%

Typical patient is female and less than 40

They are antismooth muscle antibody (ASMA) positive in 80% of cases.

ANA +ve in 10% of cases

IgG is increased in 97% of cases

They have a good response to immunosuppression and 25% have cirrhosis at presentation.

32
Q

What is type II AIH?

A

Commoner in Europe than USA.

More often seen in children and more commonly progresses to cirrhosis and less treatable.

Typically anti-liver/kidney microsomal type 1 (LKM1) antibody positive.

Anti-liver cytosol antigen type 1 (anti-LC1)

ASMA and ANA negative

33
Q

Epidemiology of AIH.

A

Predominantly affects young or middle aged women.

They are either 10-30 yo or over 40.

34
Q

Clinical features of AIH.

A

40% present with acute hepatitis and signs of autoimmune disease such as fever, malaise, urticarial rash, polyarthritis, pleurisy, pulmonary infiltrations and glomerulonephritis.

The remaining 60% present with gradual jaundice or are asymptomatic and diagnosed incidentally with signs of chronic liver disease.

Amenorrhoea is common.

35
Q

Complications of AIH.

A

Those of cirrhosis + drug therapy

36
Q

Test findings in AIH.

A

Serum bilirubin, ALT, AST and ALP increased

Hypergammaglobulinaemia

+ve for autoantibodies (ASMA or LKM1)

Anaemia

Reduced WCC

Reduced platelets

37
Q

Findings on biopsy of AIH.

A

Mononuclear infiltrate of portal and periportal areas

Piecemeal necrosis +/- fibrosis

Cirrhosis

38
Q

What is MRCP used for in AIH?

A

Helps exclude PSC if ALP is increased disproportionately

39
Q

Diagnosis of AIH.

A

Depends on excluding other diseases.

Diagnostic critera based on IgG levels, autoantibodies and histology in absence of viral disease are helpful.

Sometimes there can be an overlap with other chronic liver diseases like PBC and PSC as well as chronic viral hepatitis, this makes diagnosing more challenging.

Biopsy can confirm

40
Q

Management of AIH.

A

Immunosuppressant therapy

Liver transplantation

41
Q

Explain immunosuppressant therapy of AIH.

A

Prednisolone 30mg/d PO for 1 month and then decrease by 5mg a month to a maintenance dose of 5-10 mg/d PO.

Prednisolone can be stopped after 2 years, however relapse occurs in 50-86%

Azathioprine 50-100mg/d PO can be used as steroid sparing to maintain remission.

42
Q

Indications of liver transplantation in AIH.

A

Decompensated cirrhosis

Failure to respond to medical therapy

Even though liver transplantation is performed recurrence can happen.

43
Q

Overlap syndromes of AIH.

A

AIH-PBC

AIH-AIC (autoimmune cholangitis)

44
Q

Associations of AIH.

A

Pernicious anaemia

UC

Glomerulonephritis

Autoimmune thyroiditis

Autoimmune haemolysis

DM

PSC

HLA A1, B8 and DR3 haplotypes

Y3M - Gastro (21 decks)

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