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Primary sclerosing cholangitis (PSC) is a disease of the bile ducts that causes inflammation and obliterative fibrosis of bile ducts inside and/or outside of the liver. This pathological process impedes the flow of bile to the intestines and can ultimately lead to cirrhosis of the liver, liver failure, and other complications, including but not limited to bile duct and liver cancer. The underlying cause of the inflammation remains unknown, but elements of autoimmunity and microbial dysbiosis have been described and are suggested by the fact that approximately 75% of those with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis. The most definitive treatment for PSC is liver transplantation. ==Signs and symptoms== Many patients with PSC are asymptomatic, but a substantial proportion will have debilitating signs and symptoms of the disease.〔Tabibian JH, Lindor KD. Ursodeoxycholic acid in primary sclerosing cholangitis: If withdrawal is bad, then administration is good (right?). Hepatology. 2014 Sep;60(3):785-8.〕 These may include: * Intense pruritus (itching) * Severe fatigue (a non-specific symptom often present in liver disease) * Jaundice * Episodes of acute cholangitis (infection within the bile ducts) * Dark urine due to excess conjugated bilirubin, which is water-soluble, being excreted by the kidneys (i.e. choluria) * Malabsorption (especially of fat) and steatorrhea (fatty stool) due to biliary obstruction, leading to decreased levels of the fat-soluble vitamins, A, D, E and K. * Cirrhosis * Hepatomegaly (enlarged liver) and right upper quadrant abdominal pain * Portal hypertension, including esophageal and parastomal varices〔Tabibian JH, Abu Dayyeh BK, Gores GJ, Levy MJ. A novel, minimally-invasive technique for management of peristomal varices. Hepatology. 2015 Jun 4. doi: 10.1002/hep.27925.〕 * Hepatic encephalopathy (mental status alteration/disturbance caused by liver dysfunction and shunting of blood away from the scarred liver) 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Primary sclerosing cholangitis」の詳細全文を読む スポンサード リンク
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