Internal Medicine

Primary biliary cirrhosis

Definition of primary biliary cirrhosis

Primary biliary cirrhosis is one chronic cholestatic Disease of the liverwhich is assumed to be autoimmune.

Epidemiology / frequency

It mainly affects women over 40 years of age. They make up 90% of the patients. Every year about 5 / 100,000 people fall ill, while the prevalence is 40-80 / 100,000 people.

The primary cause is biliary cirrhosis

The autoimmune development of the disease is probably primarily caused by biliary cirrhosis. Often times she goes with other autoimmune diseases like rheumatism hand in hand.

These symptoms can indicate primary biliary cirrhosis

itching
Jaundice (yellowing of the skin and eyes)
agonizing exhaustion
Xanthoma deposition
- Elbow
- Palms
- Achilles tendon,
- Upper and lower eyelid
Dark discoloration of the skin due to melanin deposition

In many cases of primary biliary cirrhosis, however, no symptoms occur over a long period of time and only an elevated liver value (Gamma GT) during a routine examination indicates the disease.

Complications

Steatorrhea
Vitamin deficiency for fat-soluble vitamins
osteoporosis
Gallstones

Diagnosis

In the serum the increased Gamma GT in primary biliary cirrhosis, there is also the so-called AMA (A.ntimitochondrial A.antibodies) as well as antibodies against the bile ducts. If there are still doubts after evaluating the blood count, a liver puncture can confirm the diagnosis.

therapy

There is a drug available that can help reduce the time to an eventual Liver transplant can be prolonged by the excretion of Bile acids is increased. Also used are forms of therapy that are specific to your own immune system and thus slow down the progression of the autoimmune disease. A liver transplant is indicated in the late stages. Itching and vitamin deficiency can also be treated with medication.