Abstract
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are rare,
autoimmune mediated cholestatic liver diseases. Other auto-immune diseases are often
associated with PBC and PSC, and inflammatory bowel disease is present in the majority
of PSC patients. In the course of disease, chronic inflammation in the liver leads
to fibrotic restructuring and ultimately cirrhosis. The diagnosis of PBC is confirmed
serologically and PSC is diagnosed via cholangiography, whereas MRCP is preferred
over ERCP. For PBC, the first line therapy is ursodeoxycholic acid (UDCA). Prognosis
is strongly dependent on the response to UDCA. The only approved second line therapy
is obeticholic acid (OCA). Alternatively, Budesonide or Fibrates are often used off-label.
In the management of PSC, prevention and adequate treatment of bacterial cholangitis
play a major role. For both PBC and PSC novel treatments are currently being tested
in clinical trials. Disease management should address compromising symptoms like pruritus
and sicca as well as complications due to maldigestion and concomitant autoimmune
diseases. The only curative treatment available is liver transplantation and should
be considered at a MELD score of 15.
Die primär sklerosierende Cholangitis und die primär biliäre Cholangitis sind autoimmun
vermittelte cholestatische Lebererkrankungen, die unbehandelt chronisch-progredient
verlaufen und über Fibrosestadien zur Leberzirrhose führen. Dieser Beitrag gibt einen
Überblick über das diagnostische Vorgehen, die häufigen Begleiterkrankungen, die im
Behandlungskonzept zu berücksichtigen sind, und die aktuellen Therapieoptionen.
Schlüsselwörter
primär biliäre Cholangitis - primär sklerosierende Cholangitis - Cholestase - Ursodesoxycholsäure
- Obeticholsäure
Key words
primary biliary cholangitis - primary sclerosing cholangitis - cholestasis - ursodeoxycholic
acid - obeticholic acid
