Primary sclerosing cholangitis
| Primary sclerosing cholangitis | |
|---|---|
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| Cholangiogram of primary sclerosing cholangitis. | |
| Classification and external resources | |
| Specialty | Gastroenterology |
| ICD-10 | K83.0 |
| ICD-9-CM | 576.1 |
| OMIM | 613806 |
| DiseasesDB | 10643 |
| MedlinePlus | 000285 |
| eMedicine | med/3556 |
| Patient UK | Primary sclerosing cholangitis |
| MeSH | D015209 |
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 lead to cirrhosis of the liver, liver failure, and other complications, including bile duct and . The underlying cause of the inflammation remains unknown, but elements of autoimmunity and microbial dysbiosis have been described and are suggested by the observation that approximately 75% of individuals with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis. The most definitive treatment for PSC is liver transplantation, though only a fraction of individuals with PSC will ultimately require it.
Many patients with PSC are asymptomatic, but a substantial proportion will have debilitating signs and symptoms of the disease. These may include:
Primary sclerosing cholangitis is idiopathic (i.e., having, as at present, no known cause). While thought to be an autoimmune disease, it does not demonstrate a clear response to immunosuppressants. Thus, many experts believe it to be a complex, multifactorial (including immune-mediated) disorder and perhaps one that encompasses several different hepatobiliary diseases.
Data have provided novel insights suggesting:
In addition, there are longstanding, well-recognized associations between PSC and human leukocyte antigen (HLA) alleles (e.g., A1, B8, and DR3).
PSC is characterized by inflammation of the bile ducts (cholangitis) with consequent stricturing (i.e., narrowing) and hardening (sclerosis) of these ducts due to scar formation, be it inside and/or outside of the liver. The resulting scarring of the bile ducts obstructs the flow of bile, which further perpetuates bile duct and liver injury. Chronic impairment of bile flow due to blockage and dysfunctional bile transport (cholestasis) causes progressive biliary fibrosis and ultimately biliary cirrhosis and liver failure.
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