1. Epidemiology and aetiology
Chronic hepatitis implies inflammation in the liver persisting for three months or more.
Cirrhosis is a pathological term that reflects the combination of nodule formation, fibrosis and regeneration.
In recent years, there has been a progressive increase in the number of patients diagnosed, admitted to hospital and dying from cirrhosis in the UK. This increase is in part due to better recognition of chronic liver disease but the main cause is alcohol.
A study published in the Lancet showed that between 1987–91 and 1997–2001, the mortality rates from cirrhosis has increased substantially. Scotland now has one of the highest mortality rates.
It is likely also as the number of people with obesity continues to rise, that the number of people with chronic hepatitis, cirrhosis and even cancer from fatty liver will become a clinical issue.
The majority of cases of chronic hepatitis and cirrhosis that require admission to hospital are related to alcohol, viral infections (hepatitis B and C) and increasingly to non-alcoholic fatty liver disease (NAFLD).
Cirrhosis results from chronic liver damage and has many causes.
The signs and symptoms of liver disease are usually non-specific: jaundice, with pale stools and dark urine and weight loss clearly point to a hepatic cause. However, many patients with established cirrhosis present with non-specific symptoms, such as tiredness and itching.
The clinical features of cirrhosis are variable. Jaundice may be present, but might be due to non-hepatic causes and can be difficult to detect in artificial light.
In those with chronic liver disease, muscle wasting is often common and affects the face, shoulders and limbs.
Signs and symptoms
In the hands, clubbing of the fingers may be seen as well as liver palms (palmar erythema) and Dupuytren’s contractures, although the latter is not as well correlated with alcoholic liver disease as was traditionally taught. A liver flap may be seen in hepatic encephalopathy.
In the abdomen, the liver may be enlarged and is often more readily palpable in the epigastrium, because cirrhosis may be associated with shrinkage of the right lobe and hypertrophy of the left lobe. Ascites and splenomegaly may also be present, as may a para-umbilical hernia and a caput medusa.
Loss of body hair, gonadal atrophy and gynaecomastia may also be seen, although the latter may be caused by medication, such as spironolactone.
In the vascular system, always check the jugular venous pressure, as an increased JVP may point to a cardiac cause for disease. Peripheral oedema is often present, and may not be associated with cardiac disease.
Some causes of liver disease such as alcohol and iron overload may also cause a cardiomyopathy. More recently, a condition of cirrhotic cardiomyopathy where cirrhosis itself is associated with cardiac dysfunction has been recognised.
Spider naevi are also variable and may be seen in non-hepatic situations (including in healthy patients and in pregnancy). Multiple spider naevi are suggestive of alcoholic liver disease. Pigmentation of the skin is a feature of iron overload and cholestatic disease (especially when around the forehead).
Investigations should be aimed at determining the extent of liver damage and its cause.
The extent of liver damage can be established clinically and serologically. It must be stressed that the so-called liver function tests are neither liver-specific, nor tests of liver function.
The extent of liver damage and the prognosis can be assessed by the Child-Pugh score, which is dependent on ascites, encephalopathy, bilirubin and clotting, or a model for end-stage liver disease (MELD) score, which depends on the serum bilirubin, creatinine and INR (www.unos.org/ resources/MeldPeldCalculator.asp?index=98).
The cause of the liver damage can be determined by a good clinical history and appropriate blood tests and non-invasive imaging; liver histology may be helpful in selected cases.
Identifying the cause
Cirrhosis itself is not a sufficient diagnosis but a cause must be identified and appropriate treatment instituted. Even in those with established cirrhosis, treatment of the underlying cause may reduce the risk of complications and prolong survival.
In some genetic conditions such as Wilson’s disease, it is mandatory to screen family members.
The diagnosis of cirrhosis must be made on histology, which involves taking a biopsy, either percutaneously or, if factors such as thrombocytopenia, impaired clotting or ascites are present, through the transjugular route. Although the procedure is safe if guidelines are followed, there is a significant morbidity of about 2 per cent, due to haemorrhage and organ puncture. There is also a small mortality of less than one in 300, and occurs more commonly in those with hepatic malignancy than in chronic liver disease.
Cirrhosis is associated with a number of health problems and therefore it is important to monitor the patient for specific complications of cirrhosis, the underlying cause, as well as to treat the patient as a whole.
In general the patient should adopt a healthy lifestyle, including avoidance of alcohol, even where this is not the cause of the liver disease. A sensible diet should be recommended.
In general, patients should avoid NSAIDs and sedatives. Caution must be used with all medications in those with chronic liver disease.
Chronic cholestasis is associated with increased osteopenia, so clinicians should consider monitoring and, where appropriate, intervention.
Dietary salt restriction is needed in patients with ascites, but diuretics and prophylactic antibiotics are often indicated. A low-protein diet is only rarely indicated when there is no other intervention for encephalopathy.
If encephalopathy is present, treat the precipitating cause. Lactulose is the mainstay of treatment; long-term use of antibiotics, such as metronidazole or oral vancomycin, should be used with caution.
Pruritus may be a feature of cholestatic disease and responds to medication in most cases. Depending on the cause, ursodeoxycholic acid, cholestyramine, sertraline, rifampicin or naltrexone may be helpful.
Lethargy may be very severe and disproportionate to the severity of liver disease. Treatment is symptomatic. In prolonged cholestasis consider replacing fat-soluble vitamins.
Varices may develop anywhere along the gastrointestinal tract and may bleed, which could be fatal. Varices that bleed are usually located in the distal oesophagus and stomach and are asymptomatic until they bleed.
All those with cirrhosis should be offered screening, with three-yearly endoscopy.
When varices do develop, the patient should be considered for prophylactic treatment with either a programme of variceal banding or injections, or with non-specific beta-blockers, such as propranolol or carvedilol.
Patients with cirrhosis (from any cause) are at risk of developing primary liver cell cancer (hepatocellular carcinoma). Those with long-standing cirrhosis, cancer and chronic viral hepatitis are at particular risk.
Most patients should be invited to take part in a surveillance programme with six-monthly ultrasound examinations and estimation of serum fetoprotein. Those who have a developing liver cell cancer should be offered treatment.
For more resources on liver disease go to www.healthcarerepublic.com/ liverdisease