Primary sclerosing cholangitis (PSC) (synonyms: idiopathic sclerosing cholangitis; PSC; ICD-10 K83.0: cholangitis, primary sclerosing) is a chronic inflammation of the extrahepatic and intrahepatic (located outside and inside the liver) bile ducts.
PSC is an autoimmune liver disease. Other autoimmune liver diseases include autoimmune hepatitis (AIH; autoimmune hepatitis), primary biliary cholangitis (PBC, formerly primary biliary cirrhosis), and IgG4-associated cholangitis (IAC).
Sex ratio: males to females is 2-3: 1.
Peak incidence: the disease occurs predominantly between the 3rd and 5th decade of life (men: median diagnosis between the 36th and 52nd year of life).
The prevalence (frequency of disease) is 4 to 16 cases per 100,000 inhabitants.
The incidence (frequency of new cases) is approximately 1-5 cases per 100,000 inhabitants per year (in Germany) – with an increasing tendency.
Course and prognosis: In the early stages, the disease is asymptomatic. One third of all patients are symptom-free. In most cases, primary sclerosing cholangitis (PSC) is diagnosed by chance (laboratory findings). PSC is not curable. In the course of the disease, fibrotic changes (pathological proliferation of connective tissue) and consequently sclerosis (hardening) and stenosis (narrowing) of the bile ducts occur. The outflow of bile from the liver into the small intestine is no longer guaranteed due to the destruction of the extra- and intrahepatic bile ducts. Cholestasis (bile stasis) may result. Over time, the liver is damaged by toxic (poisonous) components of the stagnant bile, leading to the development of cirrhosis (liver shrinkage). Primary sclerosing cholangitis is associated with an increased risk of various malignancies (tumor diseases), especially cholangiocellular carcinoma (CCC; bile duct carcinoma, bile duct cancer). Cancer screening or early detection is of high importance in this context.
Without liver transplantation (LTx), the median survival time is between 10 and 20 years. After liver transplantation, the 10-year survival rate is about 80% – in 20% of cases the disease recurs. The risk of death due to malignancy is 40-58%.
Comorbidities: Primary sclerosing cholangitis is directly associated with rheumatic diseases. Furthermore, there is a clear association with inflammatory bowel disease (IBD): 60-80% of PSC patients also suffer from ulcerative colitis (chronic inflammatory disease of the mucosa of the rectum and possibly of the colon). of the colon (large intestine)) and 7-21% have Crohn’s disease (chronic inflammatory bowel disease that can affect the entire gastrointestinal tract (from the oral cavity to the anus)).Likewise, primary sclerosing cholangitis (PSC) is often associated with nonalcoholic fatty liver (NAFLD).
