Primary Sclerosing Cholangitis
- causes and risk factors
- signs, symptoms, and diagnosis
- pathophysiology
- treatments
- consequences and course
- the patient
- health care team
- community involvement
- resources and references
PSC is an idiopathic condition of nonmalignant, nonbacterial, chronic inflammatory fibrosis, with destruction of intrahepatic and extrahepatic bile ducts.
Causes and Risk Factors
PSC most commonly occurs in young men, often in association with ulcerative colitis.
Signs, Symptoms, and Diagnosis
The clinical spectrum of PSC is varied. Asymptomatic patients can have abnormal liver enzymes (typically alkaline phosphatase), or people can have recurring episodes of fever, chills, abdominal pain, and jaundice.
Diagnosis is made by ERCP or MRCP, showing characteristic beading of the bile ducts.
Pathophysiology
Stricturing of intra or extrahepatic ducts leads to chloestasis.
Treatments
No proven therapy works, though ursodeoxycholic acid and methotrexate are being used in some centers.
Dominant biliary strictures can be opened using ERCP.
Prophylactic antibiotics can be given to prevent recurrent cholangitis, and fat-soluble vitamins can be given.
Consequences and Course
Most people with advanced PSC eventually develop end-stage liver disease, and about one-third will develop cholangiocarcinoma.
The Patient
Health Care Team
Community Involvement
References