Liver Disease pp 237-254 | Cite as

Primary Sclerosing Cholangitis

  • Shivani Ketan Shah
  • Marina G. Silveira


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.


Primary sclerosing cholangitis Cholestasis Autoimmune liver disease Diagnosis Treatment Management 

Further Reading

  1. 1.
    Boonstra K, Weersma RK, van Erpecum KJ, Rauws EA, Spanier BW, Poen AC, et al. Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis. Hepatology. 2013;58(6):2045–55.CrossRefGoogle Scholar
  2. 2.
    Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010;51(2):660–78.CrossRefGoogle Scholar
  3. 3.
    Dave M, Elmunzer BJ, Dwamena BA, Higgins PD. Primary sclerosing cholangitis: meta-analysis of diagnostic performance of MR cholangiopancreatography. Radiology. 2010;256(2):387–96.CrossRefGoogle Scholar
  4. 4.
    Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. Primary sclerosing cholangitis – a comprehensive review. J Hepatol. 2017;67(6):1298–323.CrossRefGoogle Scholar
  5. 5.
    Lindor KD, Kowdley KV, Harrison ME. American College of G. ACG clinical guideline: primary sclerosing cholangitis. Am J Gastroenterol. 2015;110(5):646–59; quiz 60.CrossRefGoogle Scholar
  6. 6.
    Singh S, Talwalkar JA. Primary sclerosing cholangitis: diagnosis, prognosis, and management. Clin Gastroenterol Hepatol. 2013;11(8):898–907.CrossRefGoogle Scholar
  7. 7.
    Visseren T, Darwish MS. Recurrence of primary sclerosing cholangitis, primary biliary cholangitis and auto-immune hepatitis after liver transplantation. Best Pract Res Clin Gastroenterol. 2017;31(2):187–98.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Yale Traditional Internal Medicine ResidencyNew HavenUSA
  2. 2.Yale School of MedicineNew HavenUSA

Personalised recommendations