Autoimmune liver diseases are rare. In many cases, they remain undetected for a long time and are diagnosed only at a late stage. There are no exact figures and hence incidence of these diseases can only be estimated. Experts estimate that there is one case per 2000 to 4000 inhabitants in Europe affected by autoimmune liver disease.
The different autoimmune liver diseases vary with regard to incidence and their proportion with regard to gender and age.
- Has an incidence of at least 1:10000 in Europe
- Occurs in all people all over the world
- Women are thrice as often affected as men
- Appears in all ages, starting from infants up until the age of 80 or 90
- Median age at the time of diagnosis around 50
Primary biliary cirrhosis (PBC)
- Occurs in one of 5000 to 10000 persons
- 90% of affected patients are women
- the typical age of manifestation is 50 to 55 years. Young people are only very, very rarely affected.
Primary sclerosing cholangitis (PSC)
- Has an incidence of 1:10000 – 1:20000
- Men are twice as commonly affected as women
- PSC starts between the age of 30 and 50, but may also start already in childhood
- In more than every second case, PSC is combined with inflammatory bowel disease, usually colitis.