What is primary biliary cholangitis?
Primary biliary cholangitis (PBC) begins (= primary) with inflammation of the small bile ducts in the liver. The bile ducts are small channels (= ducts) that transport bile from the liver to the gallbladder and on to the small intestine. The inflammation impedes the outflow of bile from the liver, causing bile to build up there. The bile ducts are increasingly damaged by this backlog and the chronic inflammation. Over time, a latticework of scar tissue develops throughout the liver (= fibrosis). The scar tissue increasingly replaces the liver tissue. This destroys the internal structure of the liver and can lead to cirrhosis. In the case of cirrhosis, the tissue hardens and shrinks. The organ is destroyed.
Stadiums PBC
Experts divide primary biliary cholangitis into four histological stages. Liver cirrhosis is only present in the final stage of this rare autoimmune disease. Depending on the person affected, the stages can take different lengths of time. In addition, they cannot always be clearly distinguished from one another. In some cases, several stages may be present in a liver at the same time. Then some areas are more damaged than others.
What does biliary and cholangitis mean?
The word “biliary” comes from the Latin (bilis = the bile) and means “pertaining to the bile” or “belonging to the bile”. Cholangitis is an inflammation of the bile ducts.
What is the function of bile in the body?
Without the liver, there would be no bile. Our liver produces around 600 to 700 milliliters of bile every day. The body needs bile for the following purposes:
- The bile acids contained in the bile promote the digestion of fats and the absorption of fat-soluble vitamins in the intestine.
- The bile excretes so-called bile-requiring substances that the body no longer needs (for example bilirubin, excess cholesterol, degradation products of drugs).
Frequency of primary biliary cholangitis (PBC)
For unknown reasons, new diagnoses of PBC are increasing. According to the German guideline on autoimmune liver diseases (2017), PBC affects just under two to 40 out of 100,000 people, depending on the study. Primary biliary cholangitis (PBC) occurs 90 percent of the time in women. Women between the ages of 40 and 59 are most frequently affected. An increased incidence in families is possible.
Difference between primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC)
Primary biliary cholangitis affects the small bile ducts in the liver and liver cells in their vicinity. Primary sclerosing cholangitis usually affects the larger bile ducts inside and outside the liver.
What is primary biliary cirrhosis?
Primary biliary cirrhosis is the name originally used for primary biliary cholangitis. The name was changed in 2015 because PBC can now be diagnosed at a very early stage, i.e. long before cirrhosis develops. Thanks to good diagnostics and treatment options, two thirds of all PBC patients no longer develop cirrhosis at all.
Primary biliary cholangitis: causes and risk factors
It is not known why the body’s own immune system attacks the bile ducts in the liver. According to current thinking, a genetic predisposition, previously unknown toxic environmental factors or viral infections are possible triggers. PBC often occurs in conjunction with other autoimmune diseases, such as rheumatoid arthritis, inflammatory bowel disease, scleroderma, Sjögren’s syndrome, coeliac disease or androimmune thyroid disease.
PBC risk factors
Primary biliary cholangitis is not caused by alcohol consumption. However, as with all other chronic liver diseases, alcohol should be avoided. There is evidence that smoking can accelerate the progression of PBC.
Symptoms: Primary biliary cholangitis
The onset of PBC is usually gradual. About half of all those affected initially feel no symptoms.
The most common signs of primary biliary cholangitis are:
- Tiredness (fatigue),
- Feeling of pressure in the upper abdomen,
- Itching all over the body (pruritus),
- Joint pain,
- Dry mouth and eyes (sicca syndrome),
- Raynaud’s syndrome.
Other symptoms:
- Jaundice (icterus): yellowish discoloration of the skin and the whites of the eyes due to the accumulation of bile in the liver,
- Hyperpigmentation of the skin,
- Dropsy (ascites),
- varicose veins of the esophagus (esophageal varices),
- Edema: Fluid accumulates in the ankles and feet,
- Damage to the nerves (neuropathy),
- Fat deposits in the form of small yellowish nodules in the skin (xanthomas) or on the eyelids (xanthelasma),
- As the disease progresses, the itching may subside and the yellowish nodules may become smaller,
- Symptoms and complications of liver cirrhosis,
- Deficiency symptoms due to poor absorption of fats and fat-soluble vitamins (A, D, E and K),
- pale, soft, oily, foul-smelling stools because the body cannot absorb fat,
- dark urine,
- Osteoporosis due to poor absorption of vitamin D,
- Development of bruises and increased tendency to bleed due to poor vitamin K absorption,
- Enlargement of the liver and spleen,
- However, as the scarring progresses, the liver shrinks.
If left untreated, patients can die from liver failure. If the disease has occurred in your family or you experience agonizing itching and fatigue, it is advisable to seek medical advice.
Primary biliary cholangitis: diagnosis with us
In many patients, the disease is discovered during a routine blood test long before the first symptoms appear. PBC can be diagnosed relatively reliably by blood tests.
Blood values
- In PBC, the antimitochondrial antibodies (AMA) in the blood are usually elevated. These antibodies against mitochondria are the strongest indication of a disease. They are elevated in 95 percent of all PBC sufferers, but only in 1 percent of healthy test subjects.
- The alkaline phosphatase (AP) laboratory value is usually also significantly elevated. If increased AMA and increased AP occur together, a diagnosis of PBC is relatively certain.
- In addition, other liver values such as gamma-GT, bilirubin, GOT and GPT may also be elevated. However, these are not decisive for the diagnosis.
Organic tests for PBC
During a physical examination, we can feel an enlarged, firm liver in about 25 percent of cases and an enlarged spleen in about 15 percent. We use imaging techniques to examine abnormalities or blockages in the bile ducts outside the liver:
- Ultrasound examinationUltrasound examination: An ultrasound examination is a good way of determining whether, for example, there is a blockage of bile or whether the liver is also fatty.
- FibroScan (elastography): The FibroScan uses sound waves to measure how elastic or hardened the liver is.
- Magnetic resonance cholangiographyAnother imaging procedure is magnetic resonance imaging (MRI) of the bile duct system. This so-called magnetic resonance cholangiography can show changes in the bile ducts and liver.
- Magnetic resonance elastographySimilar to the FibroScan, MR elastography measures how elastic or hardened the liver is. In contrast to FibroScan, MR elastography can be used to examine the entire liver.
- Endoscopic retrograde cholangiopancreatography (ERCP): An ERCP is performed if the results of the previous imaging procedures are unclear.
In less clear-cut cases, a liver puncture (biopsy) can help to clarify the situation. We can often recognize typical PBC damage under the microscope during a tissue examination. The biopsy also helps to determine how advanced the disease is.
If necessary, further examinations are carried out to rule out other causes of liver disease.
Primary biliary cholangitis: prevention, early detection, prognosis
Primary biliary cholangitis usually progresses slowly. The first symptoms may appear after two years, but possibly also after 10 to 15 years. Some affected people become seriously ill after three to five years.
PBC progresses very differently. The course of PBC can be favorably influenced with medication. Some patients with a particularly mild course have only a few changes in their liver 20 years after the initial diagnosis. In others, the disease progresses much faster. Special blood tests can determine factors that indicate a better or worse prognosis. If there is a risk of very rapid and/or very severe damage to the liver, a liver transplant is the treatment of choice. Around two thirds of all PBC patients do not develop cirrhosis. They can grow old with their disease.
PBC: indications that the disease is progressing rapidly
- Autoimmune disease such as rheumatoid arthritis,
- rapidly worsening symptoms,
- advanced age,
- Fluid retention and other symptoms of cirrhosis,
- abnormal results in the liver function test,
- Itching subsides,
- Shrinkage of the yellow fat nodules,
Primary biliary cholangitis: treatment with medication
PBC cannot be treated causally. It remains a lifelong companion. However, the course and severity of the disease can be favorably influenced by a whole range of medications. In some cases, PBC even comes to a complete standstill. Fewer and fewer people with PBC need a transplant today.
- Medication with the active ingredient ursodeoxycholic acid promotes the excretion of bile acids. As a result, fewer complications occur and these are less serious. Unfortunately, around 30 percent of patients show no response to this treatment.
- Modern, modified forms of ursodeoxycholic acid have been used for several years if UDCA is not effective.
- Medication with the active ingredient colestyramine helps to relieve itching. The medication ensures that more bile acid, which is responsible for the itching, is excreted via the intestine.
- As this increases the deficiency of bile acid, an additional dose of the fat-soluble vitamins A, D, E and K is necessary.
If you are ill, you should avoid any further stress that impairs the function of the liver:
- Avoid alcohol,
- Avoid medications that are broken down by the liver (painkillers, sleeping pills) and
- Switch to a low-fat diet to reduce unpleasant diarrhea.
If the disease is very advanced, a liver transplant is usually necessary.