Primary biliary cirrhosis is a chronic cholestatic disease of the liver, which is assumed to be autoimmune. It mainly affects women over 40 years of age. They make up 90% of patients. Every year, about 5/100,000 people contract the disease, while the prevalence is 40-80/100,000.
Cause primary biliary cirrhosis
The disease probably has an autoimmune origin and is primarily caused by biliary cirrhosis. It is often associated with other autoimmune diseases such as rheumatism. In many cases of primary biliary cirrhosis, however, no symptoms occur over long periods of time and only an increased liver value (gamma-GT) during a routine examination indicates the disease.
- Itching
- Icterus (yellow coloration of skin and eye)
- Painful exhaustion
- Xanthoma deposition on elbows palms Achilles tendon, upper and lower eyelid
- Elbows
- Palms
- Achilles tendon,
- Upper and lower eyelid
- Dark discoloration of the skin due to melanin storage
- Elbows
- Palms
- Achilles tendon,
- Upper and lower eyelid
Complications
- Steatorrhoea
- Vitamin deficiency for fat-soluble vitamins
- Osteoporosis
- Gallstones
In serum, the elevated gamma-GT can be detected in primary biliary cirrhosis. There is also the so-called AMA (antimitochondrial antibody) and antibodies against the bile ducts. If doubts remain after evaluation of the blood count, a liver puncture can confirm the diagnosis.
Therapy
There is a drug available that can extend the time to a possible liver transplant by increasing the excretion of bile acids. Therapies that suppress the patient’s own immune system and thus slow down the progression of the autoimmune disease are also used. Liver transplantation is indicated in the late stage. Itching and vitamin deficiency can also be treated with medication.
Prognosis
The so-called bilirubin value serves as a prognostic parameter. The higher the value, the shorter the survival rate. After liver transplantation, the survival rate is good and is 70-90%.
