User:Mr. Ibrahem/Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a long-term disease in which the bile ducts inside and outside the liver become inflamed and scarred, and are eventually narrowed or blocked. Many people initially have no symptoms; however over time may develop liver disease with yellow discoloration of the skin and eyes, itching, and right upper abdominal pain. Complications may include liver failure, gallbladder cancer, bile duct cancer, bile duct infection, and fat soluble vitamin deficiencies.

The cause is unknown. In approximately 70% of cases the person has inflammatory bowel disease (IBD), most often ulcerative colitis (UC) and conversely about 7.5% of people with UC have PSC. Other risk factors include a family history of the disease and being a nonsmoker. The underlying mechanism is believed to be that of an autoimmune disease. Diagnosis may be made based a persistently elevated alkaline phosphatase and MRCP after ruling out other possible causes.

There is no cure. Efforts to slow the disease may include the use of immunosuppressants. Cholestyramine or naltrexone may be used to improve itchiness. A liver transplant is eventually required in about 40% of people, and despite transplantation the disease recurs in 25% of cases. Without a liver transplant the typical life expectancy is 10 years.

PSC is a rare, affecting about 11 per 100,000 people. Onset is usually in peoples 30s or 40s. People of Northern European ancestry are more commonly affected than those of Asian descent. Males are affected twice as often as females. The disease was first described in the mid-1800s but was not fully characterized until the 1970s.