Sammanfattning
Primary sclerosing cholangitis (PSC) is a male dominated chronic cholestatic liver disease with a strong association to inflammatory bowel disease. The gold standard for diagnosis today is MRCP which shows strictures and dilatations in the intra-and extra hepatic biliary tree. PSC is often asymptomatic but frequently progresses to symptomatic stages with abdominal pain, jaundice and bacterial cholangitis. The pathogenesis of PSC is unclear. An important differential diagnosis is IgG4 associated cholangitis which is a recently described diagnostic entity that responds well to steroids. PSC associated IBD is characterized by mild disease course high prevalence of rectal sparing and back wash ileitis and a high risk for development of colorectal dysplasia and cancer. There is no medical treatment that decreases the disease progression and patients with PSC will eventually need a liver transplantation. The most feared complication in PSC is cholangiocarcinoma which develops in up to 20 %. Once cholangiocarcinoma has developed the prognosis is poor.