Aetiologies and pathophysiologies of chronic liver disease Flashcards Preview

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Flashcards in Aetiologies and pathophysiologies of chronic liver disease Deck (40):
1

What is the flow/pathophysiology of chronic liver disease?
how would each stage present?

Causes of chronic liver disease - signs and symptoms of the casual cause
--> Recurrent inflammation and the process of fibrosis
-->Cirrhosis- compensated - stigmata of cirrhosis
--> Cirrhosis decompensated, chronic liver failure, acute on chronic liver failure - signs and symptoms of liver failure

2

List causes of chronic liver disease and cirrhosis.

Alcohol
NAFLD
Hepatitis C
Primary Biliary Cirrhosis
Autoimmune Hepatitis
Hepatitis B
Haemochromatosis
Primary Sclerosing Cholangitis
Wilsons Disease
alpha 1anti-trypsin
Budd-Chiari
Methotrexate

3

What is the difference between chronic diseases affecting the liver and chronic liver diseases?

Chronic liver diseases can lead to cirrhosis

4

What syndrome is non-alcohol fatty liver disease associated with?

Metabolic syndrome

5

What is NAFLD?

Steatohepatitis in the absence of any other cause

6

What percentage of the population have a) a fatty liver, b) NAFLD?

a) 10-20%
b) 20-30%

7

What has been suggested as a pathophysiology of NAFLD?

The 2 hit paradigm.
1st hit = excess fat accumulation
2nd hit = Intrahepatic oxidative stress
Lipid peroxidation
TNF-alpha, cytokine cascade

8

List some possible "2nd hits" in the pathophysiology of NAFLD?

Oxidative stress & lipid peroxidation
MCD diets
Pro-inflammatory cytokine release
TNFalpha
Lipopolysaccharide
Ischeamia-reperfusion injury

9

How is NASH diagnosed?

Liver biopsy

10

What is the treatment for NASH?

Weight loss & exercise

11

Primary biliary cirrhosis almost exclusively affects men or women?

Women

12

What is the clinical presentation of primary biliary sclerosis?

Middle aged women (f:m 10:1)
Usually asymptomatic/ Incidental
Symptoms
Fatigue
Itch without rash
Xanthesalma and xanthomas
Complications

13

How is PBC diagnosed?

2of 3:
Positive AMA
Cholestatic LFTs
Liver Biopsy

14

What is the treatment for PBC?

Urseo deoxycholic acid
Urseo deoxycholic acid is a bile salt
Brings more water into the bile duct, flushes away toxic bile salts reducing damages
Does not cure the disease but slows its progression

15

What are the outcomes of PBC?

Most will not develop symptoms in their life time
The majority with PBC symptoms do not develop liver failure
Itch can be particularly problematic.
Many developing liver failure will be unfit for transplant
Still a common cause of liver transplantation

16

Does autoimmune hepatitis affect women or men more frequently?

Women

17

What percentage of patients with autoimmune hepatitis develop cirrhosis?

40%

18

What is the classic presentation of autoimmune hepatitis?

A jaundiced teenage girl

19

What are the extrahepatic manifestations associated with type 1 autoimmune hepatitis?

Autoimmune thyroiditis, graves disease, chronic UC
Less commonly with RA, pernicious anemia, systemic sclerosis, ITP, SLE

20

How do 40% of patients with type 1 autoimmune hepatitis present?

with acute onset of symptoms similar to toxic hepatitis or acute viral hepatitis

21

What is the clinical presentation of autoimmune hepatitis?

Hepatomegaly
Jaundice
Stigmata of chronic liver disease
Splenomegaly
Elevated AST and ALT
Elevated PT
Non-specific symptoms: malaise, fatigue, lethargy, nausea, abdominal pain, anorexia

22

How is autoimmune hepatitis diagnosed?

Elevated AST and ALT
Elevated IgG
Rule out other causes:
Wilsons disease
Alpha 1 antitrypsin deficiency
Viral hepatitis (A, B, C)
Drug induced liver disease (alcohol, minocycline, nitrofurantoin, INH, PTU, methyldopa, etc)
NASH
PBC, PSC, autoimmune cholangitis

Presence of autoimmune antibodies
Liver biopsy

23

What is a hallmark histological finding of autoimmune hepatitis?

Interface hepatitis

24

What is the treatment for autoimmune hepatitis?

Corticosteroids
Azathioprine
Children: azathioprine or 6MP
Combination Therapy
Prednisone + Azathioprine
Prednisone: start at 30mg daily and taper down to 15mg at week 4, then maintain on 10mg daily until therapy endpoint
Azathioprine 50-100mg daily

25

What is the prognosis of autoimmune hepatitis?

40% of all pts with AIH develop cirrhosis
54% develop esophageal varices within 2 years
Poor prognosis if has presence of ascites or hepatic encephalopathy
13-20% of patients can have spontaneous resolution
Of patients who survive the most early and active stage of disease, approximately 41% of them develop inactive cirrhosis.
Of patients who have severe initial disease and survive the first 2 years, typically survive long term.

26

How does primary sclerosing cholanagitis present clinically?

As recurrent cholangitis

27

How is primary sclerosing cholangitis diagnosed?

Imaging of the biliary tree

28

What is the treatment for primary sclerosing cholangitis?

maintain bile flow
monitor for cholangiocarcinoma and colo-rectal cancer

29

What does haemochromatosis cause?

Cirrhosis
cardiomyopathy
Pancreatic failure

30

What is the treatment for haemochromatosis?

Venesection

31

What is the treatment for Wilson's disease?

copper chelation drugs

32

How is Budd chiari diagnosed?

U/S visualisation of hepatic veins

33

How is Budd chiari treated?

recanalization or TIPS

34

What is methotrexate?

Drug used to treat Rheumatoid arthritis and psoriasis

35

What is the effect of methotrexate on the liver?

Dose dependent liver toxin
Progressive fibrosis

36

What are the symptoms of liver fibrosis caused by methotrexate?

There are no clinical signs
Have to monitor the fibrosis

37

What is the treatment for the fibrosis caused by methotrexate?

Stop the drug

38

What is cardiac cirrhosis?

Cirrhosis secondary to high right heart pressures:
Incompetent tricuspid valve
Congenital
Rheumatic fever
Constrictive pericarditis

39

What is the clinical presentation of cardiac cirrhosis?

CCF, with too much ascites and or liver impairment

40

What is the treatment for cardiac cirrhosis?

Treat the cardiac condition

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