Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the <a href="/facts/Liver/Rtp4Fk2b">liver</a> and <a href="/facts/Gallbladder/RPdkQzQM">gallbladder</a> characterized by <a href="/facts/Inflammation/Ura86MIy">inflammation</a> and scarring of the <a href="/facts/Bile_duct/hBFPcYkS">bile ducts</a>, which normally allow <a href="/facts/Bile/D15BG2gq">bile</a> to drain from the gallbladder. Affected individuals may have no symptoms or may experience signs and symptoms of liver disease, such as <a href="/facts/Jaundice/TCyoLMJU">jaundice</a>, <a href="/facts/Pruritus/MBWq6SPc">itching</a>, and abdominal pain.
The bile duct scarring that occurs in PSC narrows the ducts of the <a href="/facts/Biliary_tract/NJPQ5oy4">biliary tree</a> and impedes the flow of bile to the <a href="/facts/Duodenum/IAIgHBmq">duodenum</a>. Eventually, it can lead to <a href="/facts/Cirrhosis/bkLuLvCD">cirrhosis</a> of the liver and <a href="/facts/Liver_failure/AB5ocI30">liver failure</a>. PSC increases the risk of various cancers, including <a href="/facts/Hepatocellular_carcinoma/lE0P5CqE">liver cancer</a>, <a href="/facts/Gallbladder_carcinoma/sDfkTUgc">gallbladder carcinoma</a>, <a href="/facts/Colorectal_cancer/nV592sW7">colorectal cancer</a>, and <a href="/facts/Cholangiocarcinoma/N5twpETV">cholangiocarcinoma</a>. The underlying cause of PSC is unknown. Genetic susceptibility, <a href="/facts/Autoimmunity/5cASmcfI">immune system dysfunction</a>, and <a href="/facts/Dysbiosis/nq80zc9d">abnormal composition of the gut flora</a> may play a role. This is further suggested by the observation that around 75% of individuals with PSC also have <a href="/facts/Inflammatory_bowel_disease/RuxcPW5A">inflammatory bowel disease</a> (IBD), most often <a href="/facts/Ulcerative_colitis/BIkyFzRj">ulcerative colitis</a>.
No effective medical treatment for primary sclerosing cholangitis is known. Its most definitive treatment is a <a href="/facts/Liver_transplantation/0noMXT50">liver transplant</a>, but disease recurrence can occur in 25–30% of cases. For patients unable or unwilling to receive a transplant, therapy primarily focuses on relieving symptoms, rather than stopping disease progression. If the sclerosing cholangitis is a secondary effect of a different disease, treatment is directed towards the underlying cause.
PSC is a rare disease and most commonly affects people with IBD. About 3.0 to 7.5% of people with ulcerative colitis have PSC, and 80% of people with PSC have some form of IBD. Diagnosis usually occurs in people in their 30s or 40s. Individuals of Northern European ancestry are affected more often than people of Southern European or Asian descent. Men are affected more often than women. The disease was initially described in the mid-1800s, but was not fully characterized until the 1970s with the advent of improved medical-imaging techniques such as <a href="/facts/Endoscopic_retrograde_cholangiopancreatography/BpfeWhVP">endoscopic retrograde cholangiopancreatography</a>.

Primary sclerosing cholangitis open-in-new

Primary sclerosing cholangitis