Cirrhosis

Pathogenesis:

Cirrhosis results from the necrosis of liver cells followed by fibrosis and nodule formation

The liver architecture is diffusely abnormal and this interferes with liver blood flow and function

This derangement produces the clinical features of portal hypertension and impaired liver cell function

Causes of cirrhosis:

Alcohol (most common cause in the West)

Hepatitis B ± D (most common cause worldwide)

Hepatitis C

Biliary cirrhosis

Autoimmune hepatitis

Hereditary haemochromatosis

Budd-Chiari syndrome (hepatic vein obstruction)

Wilson’s disease

Drugs (e.g. Methotrexate)

α₁-Antitrypsin deficiency

Cystic fibrosis

Intestinal bypass operations for obesity

Galactosaemia

Glycogen storage disorders

Pathology:

The characteristic features of cirrhosis are regenerating nodules separated by fibrous septa and loss of the normal lobular architecture within the nodules. Two types of cirrhosis have been described:

Micronodular cirrhosis:

Regenerating nodules are usually < 3mm in size and liver is involved uniformly

Often caused by ongoing alcohol damage or biliary tract disease

Macronodular cirrhosis:

The nodules are of variable size and normal acini may be seen within the larger nodules.

Often seen following previous hepatitis, such as HBV infection

Investigations to determine type:

Viral markers

Serum Autoantibodies

Serum Igs

Serum copper and α₁-Antitrypsin deficiency (should always be done in young cirrhotics)

Serum iron, total iron-binding capacity (TIBC) and ferritin (to exclude hereditary haemochromatosis)

Cirrhosis

Investigations to assess severity:

LFTs:

Serum albumin is the best indicator of liver function. The outlook is poor with a level < 25g/L

The PT is prolonged commensurate with the severity of the liver disease

Biochemistry can be normal, depending on the severity of cirrhosis

In most cases, there is at least a slight elevation in the serum ALP and aminotransferases

In decompensated cirrhosis, all biochemistry is deranged

Serum electrolytes:

A low sodium indicates severe liver disease because of dilution secondary to a defect in free water clearance or to excess diuretic therapy

Other:

Serum alpha-fetoprotein is a useful screening test for HCC

Imaging:

Ultrasound:

Can demonstrate changes in size and shape of the liver

Fatty change and fibrosis produce a diffuse increased echogenicity

In established cirrhosis, there may be marginal nodularity of the liver surface and distortion of the arterial vascular architecture

It is useful in detecting HCC

CT scan

Barium swallow:

Can detect varices

Liver biopsy:

This is necessary to confirm the severity and type of liver disease

Management:

Mx is that of the complications seen in decompensated cirrhosis

Pts should have six-monthly alpha-fetoprotein measurements to detect the development of a HCC as early as possible

There is no treatment that will arrest or reverse the cirrhotic changes

Alcohol should be avoided, although if the cirrhosis is not due to alcohol, small amounts are probably not harmful

Course and prognosis:

This is very variable and is dependent on a number of factors

Development of any complication usually worsens the prognosis

In general, the 5yr survival rate is ~50%