- The most common PBC symptoms are itch & fatigue.
- ~60% of PBC patients are asymptomatic at time of diagnosis.
- Not everyone with PBC will experience all, or any symptoms.
- Severity of symptoms do not correlate with severity of disease.
- Patients can take an active role in the management of their symptoms.
- Treatments & self-care practices have been shown to improve symptoms.
Disease Specific Symptoms of PBC
PBC symptoms, the most common ones being itch and fatigue, often increase and decrease in cycles. Long-standing patients with PBC will know that there are good spells and not so good spells. Within the good spells, we can have bad days and in the bad spells we can still have good days.
Symptoms Associated with Advanced Liver Disease
Whilst both pruritus and fatigue can occur at any point in the course of PBC, and do not correlate with severity of disease or with progression, there are a number of symptoms that can affect PBC patients that are associated with a more advanced liver disease.
PBC may progress to cirrhosis, this implies that there is significant scarring (fibrosis) in the liver with the formation of nodules. Whatever the cause, cirrhosis may lead to a number of complications that need to be monitored.
Many people who have cirrhosis will remain well with no complications or side-effects; this is termed Compensated Cirrhosis. It should be noted that varices and even liver cell cancer can develop if you have compensated cirrhosis, and you will need to be monitored for these complications.
The term Decompensated Cirrhosis means that the patient is developing complications of the cirrhosis. These may include varices, ascites, hepatic encephalopathy, ankle swelling, or muscle loss. Decompensation is sometimes an indication to consider transplantation although decompensation may be triggered by another event, e.g. an infection.
The main conditions associated with cirrhosis are explained below:
Hepatocellular Carcinoma (HCC)
Patients with cirrhosis, of whatever cause, are at higher risk of developing cancer of the liver (hepatocellular carcinoma but also known as hepatoma). This affects men more than women. People with cirrhosis will be asked to undergo regular blood tests and ultrasound examinations (usually every 6 months): if detected early, the cancers can often be treated or even cured. There are now many approaches to treatment of liver cancer.
Hepatic Encephalopathy (HE)
When the liver fails to clear the toxins from the bowel, the toxins will enter the circulation and may cause hepatic encephalopathy (HE). HE may present in many ways, varying from mild drowsiness or confusion to coma. There may be difficulty is doing simple tasks, such as tying shoelaces or writing. Sleep patterns may be disturbed.
Many factors can trigger the development or worsening of HE: such factors include infection, bleeding from the gastrointestinal tract, constipation, and some medications. Diagnosis may be made clinically (there is a characteristic flap sometimes) and by EEG (where the brain waves are recorded). Treatment is by treating any precipitating factor, lactulose (to ensure you have at least two soft bowel motions a day) and sometimes drugs.
Ascites is the build-up of fluid in the abdomen. As liver function deteriorates and scarring increases, there is less production of a protein called albumin by the liver and increased resistance to blood flow to the liver. These factors result in fluid accumulating in the abdomen.
Pedal oedema (ankle swelling)
There are many causes of ankle swelling (pedal oedema). A low level of albumin, seen in advanced liver disease is a cause of ankle swelling. Treatment for both ascites and pedal oedema is usually diuretics and a low salt-diet. Sometimes, this regimen is ineffective so other interventions are required: these may include a shunt (‘TIPS’) or an abdominal drain.
Varices are dilated veins which can develop as a consequence of advanced scarring and cirrhosis, whatever the cause of the cirrhosis. Whilst normally associated with cirrhosis, varices can occur in PBC patients before cirrhosis has developed. In the GI (gastrointestinal) tract, varices can occur anywhere between the mouth and the anus but tend to form at the lower end of the gullet (oesophageal varices) and in the stomach (gastric varices).
Varices cause no problems until they start to bleed. Not everyone who has PBC, or indeed cirrhosis, develops varices, and not everyone who has varices experiences a bleed. When they do bleed, however, the consequences are serious and potentially fatal so, they need to be detected and treated before this happens.
Those with advanced scarring or with cirrhosis will be asked to have an endoscopy to look for varices. If the varices are significant, the patient will usually be offered treatment to prevent bleeding: either pharmacological, banding or both.
Jaundice is the result of a build-up of bilirubin in the blood and leads to a yellow colour in the eyes, and skin. Jaundice may be seen in many liver and non-liver conditions. Often it is associated with dark-yellow urine and sometimes pale stools. With mild jaundice, the yellow may be difficult to detect and a blood test is the only reliable way of detecting jaundice.
Most of the bilirubin comes from the haemoglobin in red cells: this is broken down by the liver and excreted in the bile and urine. When there is liver damage or obstruction to bile flow, bilirubin levels rise, and jaundice becomes evident.
In PBC, increasing jaundice is a sign that the liver is running into difficulties: there are many causes of jaundice and when a patient with PBC starts to get jaundice, many causes need to be considered before attributing the jaundice to advanced PBC.
Portal hypertension can be a consequence of cirrhosis. This describes an increase in pressure in the portal vein (which carries blood from the bowel and spleen into the liver). The increase in pressure could be a consequence of a cirrhotic liver not being able to allow that blood to flow easily into the liver. Portal hypertension can elevate the risk of variceal bleeds, so monitoring and treatment is advised.
Interaction with the Foundation should never replace your consultation with your clinician. Our aim is to enhance your knowledge in order to aid your consultation. If you have any concerns about your PBC, symptoms, treatment, or any aspect at all of you living with PBC then please do contact us directly and we shall help wherever we can.