Primary sclerosing cholangitis (PSC) is a rare, chronic cholestatic liver disease characterized by progressive multifocal strictures of the intra- and extrahepatic bile ducts. Patients are often asymptomatic on presentation, but common symptoms include pruritus, fatigue, and abdominal pain. During the course of the disease, many patients develop recurrent cholangitis, biliary cirrhosis, and end-stage liver disease. No effective medical therapy for PSC is currently available, but ursodeoxycholic acid is commonly used in practice. Ultimately, many patients with PSC may require transplant, after which recurrent disease is a risk. Complications including bacterial cholangitis, fat-soluble vitamin deficiency, metabolic bone diseases, and development of hepatobiliary or colon cancers can occur. In this chapter, we describe a case of primary sclerosing cholangitis in an asymptomatic patient. We discuss the differential diagnosis, evaluation, management, and treatment of PSC.
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