137 - Patterns of liver injury 3: Cirrhosis and Portal Hypertension Flashcards
Autoimmune hepatitis
1
2 a, b, c, d, e
1) Aggressive form of chronic hepatitis
2) Features of autoimmune conditions:
a) Mostly female (80%)
b) Genetic predisposition
c) Association with other autoimmune diseases
d) Presence of serum autoantibodies
e) Therapeutic response to immune suppression.
Progression rate of autoimmune hepatitis to cirrhosis
85% over 5 years
Types of autoimmune hepatitis
1
2
- Type 1 (most common) occurs in adult women and associated with anti-smooth muscle antibodies (ASMA)
- Type 2 occurs in children and teenagers associated with anti-liver kidney microsome (LKM-1)
Patterns of injury of autoimmune hepatitis
1
2
- Severe early phase with destruction of liver cells: presents as acute hepatitis clinically and pathologically.
- Fibrosis (scarring) develops rapidly in the areas of collapse (unlike most cases of chronic viral hepatitis, where fibrosis accumulates slowly).
How is the fibrosis in autoimmunehepatitis unlike other cases of chronic hepatitis?
Fibrosis develops rapidly in areas of collapse (unlike most cases of chronic viral hepatitis, where fibrosis accumulates slowly)
Definition of cirrhosis
1
2
3
• Conversion of the liver into nodules of regenerating hepatocytes surrounded by bands of fibrous (scar) tissue
• Diffuse (involves the whole liver)
• Vascular shunting
An anatomical term describing the end-state of many chronic liver conditions
End-stage chronic liver failure
Liver can no longer perform its functions
Differences between end-stage liver failure and cirrhosis
1
2
The two are often associated, however:
• Not all patients with ESCLF have cirrhosis (e.g. primary sclerosing cholangitis)
• Not all patient with cirrhosis have ESCLF (treated AIH, suppressed HBV or cured HCV may be clinically stable even when cirrhosis is present)
Gross appearance of a cirrhotic liver
1
2
3
1) Coarsely nodular surface (nodules up to 2cm)
2) Nodules are well-demarcated
3) Nodules can be green from marked cholestasis
Adult causes of cirrhosis 1 2 3 4 5 6 7
- Alcoholic liver disease
- Non-alcoholic steatohepatitis
- Chronic viral hepatitis B and C
- Autoimmune hepatitis
- Chronic biliary disease – primary sclerosing cholangitis and primary biliary cirrhosis
- Metabolic – Wilson’s disease (genetic copper toxicosis) Haemochromatosis, alpha-1-antitrypsin deficiency
- Drugs – methotrexate, methyldopa
Variables in cirrhosis appearance
1) Nodule size
2) Pattern of scarring
3) Degree of liver tissue destruction (parenchyma extinction).
4) Thrombosis of vessels especially portal veins is common and this varies in degree and the size of vessel involved.
What can differences in cirrhosis appearance tell us?
Little about the cause of the cirrhosis
More recent concept of cirrhosis pathogenesis
1
2
3
- In chronic liver disease, hepatocellular death due to the direct effect of the insult itself is relatively unimportant, as these cells can be replaced by mitotic division of hepatocytes
- Scars and fibrous septa develop in the areas where the hepatocytes have dropped out as a result of inflammatory injury to the portal veins.
- Progression to cirrhosis is driven by “parenchymal extinction” caused by vascular inflammation and thrombosis.
Which types of cirrhosis have the greatest risk of hepatocellular carcinoma?
Cirrhosis due to alcohol, chronic viral hepatitis B and C, haemochromatosis
Amount of hepatic blood flow from portal vein
2/3
