Primary sclerosing cholangitis
The etiology of PSC is unknown. PSC 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 of age, 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 it is most commonly detected by incidental liver test abnormalities, particularly elevation in alkaline phosphatase (ALP). 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.
K83.01 – Primary sclerosing cholangitis
197441003 – Primary sclerosing cholangitis
Differential Diagnosis & Pitfalls
- Infectious or ischemic cholangiopathy
- Hepatic metastases (see liver cancer)
- Portal hypertensive cholangiopathy (see portal hypertension)
- Surgical biliary trauma
- Biliary colic
- Eosinophilic cholangitis
- IgG4-related sclerosing cholangitis (see IgG4-related disease)