Primary biliary cirrhosis is a rare chronic liver disease. It is now known as primary biliary cholangitis.
What is primary biliary cirrhosis?
Primary biliary cirrhosis is the former name for a rare liver disease. However, since the term “primary biliary cirrhosis” was considered misleading, the disease was renamed primary biliary cholangitis (PBC). In this way, the disease can often be diagnosed before liver cirrhosis develops.
Due to modern examination and therapy methods, around 66 percent of all patients no longer develop cirrhosis. In addition, those affected are often unsettled by the term “cirrhosis”. In 2014 and 2015, medical professional associations in Europe and America made the decision to adapt the new term “primary biliary cholangitis” to the liver disease. See healthknowing for What is the Definition of PI.
In the meantime, the WHO (World Health Organization) is also examining a renaming. Primary biliary cirrhosis or primary biliary cholangitis primarily affects women. Around 90 percent of all sufferers are women. The disease primarily begins in the small bile ducts and then spreads to the entire liver tissue, which in turn can result in scarring.
However, cirrhosis of the liver only becomes apparent in the final stages of the disease. The exact global extent of primary biliary cirrhosis is unclear. According to estimates, between 4,000 and 12,000 Germans suffer from liver disease in Germany, which mainly manifests itself in middle-aged people between 40 and 60 years of age.
Causes
Because antimitochondrial antibodies occur in more than 95 percent of all patients, medicine classifies primary biliary cirrhosis as an autoimmune disease. We speak of an autoimmune disease when the body’s own defense system is no longer able to distinguish between the body’s own and foreign substances. In the case of PBC, the mitochondria in the body’s own cells are attacked.
Autoantibodies are produced which are directed against the E2 subunit of the pyruvate dehydrogenase complex. This is the enzyme dihydrolipoyl transacetylase. However, it is still controversial whether other factors are also responsible for the onset of primary biliary cirrhosis.
Genetic and hormonal influences are discussed. The same applies to bacterial, viral or fungal infections, the use of certain drugs or environmental factors. It is also unclear what effects primary biliary cirrhosis has on pregnant women.
Symptoms, Ailments & Signs
Doctors divide primary biliary cirrhosis into four different stages. In stage I, the epithelium of the bile ducts is destroyed, while in stage II, there is proliferation of the bile ducts. This leads to the formation of pseudobile ducts. Stage III is when fibrosis of the portal fields with necrosis occurs and the bile ducts progressively disappear.
In the fourth and last stage, cirrhosis of the liver finally appears and the organ takes on a greenish colour. The symptoms of primary biliary cirrhosis begin insidiously. About 70 to 90 percent of patients feel tired and exhausted. Itching, a disease of the thyroid gland such as Hashimoto’s thyroiditis, dry mucous membranes, joint problems and complaints that resemble rheumatism are not uncommon.
Around 20 percent of all patients suffer from fat deposits in the area of the inner corners of the eyes. Other possible symptoms can be fatty stool and a lack of vitamins. Urinary tract infections are also not uncommon in female patients. In the late stage of primary biliary cirrhosis, typical cirrhotic complications such as esophageal varices, fundus varices, ascites (water belly), liver cancer and brain dysfunction occur.
Diagnosis & course of disease
If primary biliary cirrhosis is suspected, laboratory tests are carried out. More than 90 percent of all PBC patients have antimitochondrial antibodies (AMA) in their blood. This finding alone can serve as proof of the presence of primary biliary cirrhosis.
Furthermore, the general laboratory values are also above normal values and indicate inflammation or congestion of the bile ducts. If the laboratory tests do not provide precise evidence, a liver biopsy is performed. The diagnosis can be confirmed by removing liver tissue.
In addition, it is important to differentiate primary biliary cirrhosis from other autoimmune diseases. In earlier years, PBC patients had an average life expectancy of about twelve years. During this period, however, the disease was usually not discovered until it was in its final stages.
Basically, the course of PBC turns out to be very different from person to person. If the course of the disease is mild, there are usually only minor changes, while in other cases a rapid course of the disease can be expected. However, two out of three PBC patients no longer develop life-threatening liver cirrhosis.
Complications
Patients with this disease suffer from various liver problems. If this disease is not treated, it can even lead to the death of the patient in the worst case. For this reason, this condition must be treated by a doctor in any case. Those affected primarily suffer from necrosis.
This disease also leads to cirrhosis of the liver and eventually to destruction of the liver. Those affected suffer from itching and also from jaundice. The mucous membranes dry out and there are problems in the joints, so that the patients also suffer from restricted mobility. Without treatment, unpleasant side effects such as fatty stool and diseases of the urinary tract are encouraged.
In the worst case, liver cancer also occurs, in which case transplantation of the organ is usually necessary. The earlier the disease is diagnosed, the better the chances of a complete cure. In severe cases, patients are dependent on a liver transplant in order to continue to survive. The disease is treated with medication.
When should you go to the doctor?
Itching and skin changes indicate primary biliary cirrhosis and should be clarified as soon as possible by your family doctor or a dermatologist. As the disease progresses, symptoms such as tiredness, joint problems or jaundice can also occur, which must also be examined by a doctor. If liver or spleen problems occur, it is best to consult a doctor immediately. If cirrhosis of the liver occurs, the emergency doctor must be called. Primary biliary cirrhosis occurs in women in 90 percent of cases.
It usually becomes noticeable between the ages of 40 and 60. A doctor should be consulted if these risk factors apply and the symptoms mentioned occur. Then it is best to see your family doctor or a specialist in internal medicine. Depending on the symptoms, specialists in liver diseases as well as gastroenterologists and dermatologists can also be consulted. The actual treatment takes place in a specialist clinic. Children should be presented to the pediatrician if the above symptoms and signs appear.
Treatment & Therapy
Primary biliary cirrhosis is treated by administering ursodeoxycholic acid (UDC). The patient takes this as a tablet for the rest of his life. In the early stages of PBC, slowing or stopping the disease is possible with this drug.
On the other hand, the benefit of immunosuppressants that were administered in the past is controversial. However, they usually only make sense if autoimmune hepatitis is also present. If liver cirrhosis develops despite treatment, a liver transplant may be necessary. This comprehensive intervention can cure PBC in 75 percent of all cases.
Prevention
Prevention of primary biliary cirrhosis is not possible. The exact causes of the disease are still being researched.
Aftercare
Primary biliary cirrhosis requires regular follow-up examinations for constant monitoring by the doctor. The patients should definitely keep the appointments, because the exact diagnosis is also an essential element in the follow-up treatment. In this way, a relapse can be detected early. The course of the disease can be checked precisely through the controls as part of the aftercare.
According to the routine, check-ups are recommended every three to six months. If the laboratory values deteriorate after the actual therapy, further examinations are scheduled. Patients should not postpone the examinations, but consult the doctor in good time. Regardless of whether a liver transplant has taken place or not, it is essential for those affected to pay attention to other symptoms of the disease.
Increased fatigue and frequent itching indicate a deterioration. A regular daily rhythm helps to find a certain balance. Sufficient breaks and sleep phases must be observed so that the patient’s condition improves. After liver transplantation, long-term care is advisable. This is available in the relevant transplant centers. In addition to the recommendations of the doctors, the mental support from family and friends is also very valuable for those affected.
You can do that yourself
Unfortunately, there is no therapeutic approach for this autoimmune disease that can cure the disease. The prescribed drugs can not only alleviate the symptoms, but also delay the point in time until cirrhosis and thus the necessary liver transplant. They should therefore be taken regularly. Medical checks should also be carried out regularly.
Due to possible mental problems that such a diagnosis can cause, an accompanying psychotherapy is recommended, in which the fears and insecurities that arise are discussed appropriately and can thus be overcome. Talking to people who are also affected is also helpful. There is a liver self-help group and a patient group at the Erlangen Liver Center. There is even a Facebook group for people with primary biliary cirrhosis. If you research here on the Internet, you will quickly find what you are looking for.
A reliably working immune system is also important, especially in the case of an autoimmune disease. There are many ways to strengthen the immune system. Detoxification measures such as intestinal cleansing or detoxification cures supplement dietary measures such as a low-fat diet that relieves the liver. Above all, animal fats should be avoided and high-quality omega-3-rich vegetable oils should be used instead. Omega-3 fatty acids can be found, for example, in linseed oil or walnut oil.
Furthermore, people with liver disease should avoid stress. Instead, a lot of sleep and rest, but also a lot of exercise such as hiking, walking or swimming are indicated.
