Primary sclerosing cholangitis
The etiology of PSC is unknown; it is associated with inflammatory bowel disease and is believed to be caused by a combination of genetic and environmental factors. PSC is most commonly diagnosed in patients around 40 years old, and 60% of patients are male. The highest incidences of PSC are in the United States and Northern Europe. Many patients are asymptomatic at time of diagnosis and early in the disease process, and PSC is detected by incidental liver test abnormalities. Common symptoms include pruritus, fatigue, and jaundice.
There is no specific approved therapy available for PSC and no cure besides liver transplantation. Goals of therapy focus on symptom management. There is mixed literature on the utility of ursodeoxycholic acid (ursodiol) and more recently obeticholic acid (US Food and Drug Administration [FDA] approved in 2016, unclear long-term benefit). However, the natural course of PSC is toward liver failure, with 40% of patients requiring a liver transplant. Prognosis for patients who have undergone liver transplantation is excellent, and a good quality of life should be expected after recovery, although disease recurrence is possible.
For more information, see OMIM.
K83.0 – Cholangitis
197441003 – Primary sclerosing cholangitis
- Infectious or ischemic cholangiopathy
- Chronic bacterial cholangitis
- Diffuse intrahepatic metastases
- Portal hypertensive cholangiopathy
- Recurrent pancreatitis
- Recurrent pyogenic cholangitis
- Surgical biliary trauma
- Eosinophilic cholangitis
- Intra-arterial chemotherapy
- IgG4-related sclerosing cholangitis (IgG4-related disease)