| LiverAtlas Disease ID | HuLDi00155 |
| Name | Primary sclerosing cholangitis |
| Description | | Xref: |
| Definition | 1.A chronic, autoimmune inflammatory liver disorder characterized by narrowing and scarring of the lumen of the bile ducts. It is often seen in patients with ulcerative colitis. Signs and symptoms include jaundice, fatigue, and malabsorption. It may lead to cirrhosis and liver failure. | Xref: UMLS2011AA:C0566602 |
| 2.Recurrent or persistant obstructive jaundice, frequently with ulcerative colitis, due to extensive obliterative fibrosis of the bile ducts. | Xref: UMLS2011AA:C0566602 |
| 3.Primary sclerosing cholangitis is a chronic liver disease caused by progressive inflammation and scarring of the bile ducts of the liver. The inflammation impedes the flow of bile to the gut, which can ultimately lead to liver cirrhosis, liver failure and liver cancer. The underlying cause of the inflammation is believed to be autoimmunity; and more than 80% of those with PSC have ulcerative colitis. The definitive treatment is liver transplantation. | Xref: URL |
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| Synonym | PSC | EXACT | Xref: Clinical Hepatology, 2004:Page 437 |
| Primary sclerosing cholangitis | EXACT | Xref: UMLS2011AA:C0566602 |
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| Xref | Clinical Hepatology,2004.Page 437 |
| Schiff's Diseases of the LIVER,10ed:Chapter 23 |
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| Genetics Relation | Gene/Protein/Pathway |
| Gene:AIRE |
Gene:FAS | Gene:C3 | Gene:C4A | Gene:CD247 | Gene:CD4 | Gene:CD8A | | more... |
