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Liver Disease pp 221-235 | Cite as

Primary Biliary Cholangitis

  • Andrew R. Scheinberg
  • Cynthia LevyEmail author
Chapter

Abstract

Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is the most common autoimmune liver disease. PBC is characterized as a chronic inflammatory autoimmune cholestatic liver disease where immune-mediated injury to biliary epithelial cells leads to cholestasis and fibrosis, and if left untreated, PBC will progress to end-stage liver disease. The disease affects predominantly middle-aged women, with data suggesting 1 in 1000 women over the age of 40 live with PBC. Importantly, clinical presentation ranges from being completely asymptomatic to significantly affecting the patient’s quality of life via pruritus, fatigue, abdominal pain, and sicca symptoms (dry mouth, dry eyes) in addition to liver-related complications. In this chapter, we describe the classic presentation of an asymptomatic patient with a cholestatic pattern of liver injury. We discuss the initial work-up and differential diagnosis for this pattern, the diagnostic criteria for PBC, and specific treatment, with an emphasis on early referral to a liver specialist to ensure the proper management of this progressive liver disease.

Keywords

Cholestasis Treatment Ursodeoxycholic acid Obeticholic acid Risk stratification Pruritus 

Further Reading

  1. 1.
    Boberg KM, Chapman RW, Hirschfield GM, Lohse AW, Manns MP, Schrumpf E. Overlap syndromes: the international autoimmune hepatitis group (IAIHG) position statement on a controversial issue. J Hepatol. 2011;54(2):374–85.CrossRefGoogle Scholar
  2. 2.
    Carey EJ, Ali AH, Lindor KD. Primary biliary cirrhosis. Lancet. 2015;386(10003):1565–75.CrossRefGoogle Scholar
  3. 3.
    Corpechot C, Carrat F, Poujol-Robert A, Gaouar F, Wendum D, Chazouillères O, et al. Noninvasive elastography-based assessment of liver fibrosis progression and prognosis in primary biliary cirrhosis. Hepatology. 2012;56(1):198–208.CrossRefGoogle Scholar
  4. 4.
    Dufour DR, Lott JA, Nolte FS, Gretch DR, Koff RS, Seeff LB. Diagnosis and monitoring of hepatic injury. II. Recommendations for use of laboratory tests in screening, diagnosis, and monitoring. Clin Chem. 2000;46(12):2050–68.PubMedGoogle Scholar
  5. 5.
    Hirschfield GM, Beuers U, Corpechot C, Invernizzi P, Jones D, Marzioni M, et al. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017;67(1):145–72.CrossRefGoogle Scholar
  6. 6.
    Kwo PY, Cohen SM, Lim JK. ACG clinical guideline: evaluation of abnormal liver chemistries. Am J Gastroenterol. 2016;112:18.CrossRefGoogle Scholar
  7. 7.
    Lammers WJ, van Buuren HR, Hirschfield GM, Janssen HLA, Invernizzi P, Mason AL, et al. Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study. Gastroenterology. 2014;147(6):1338–49. e5.CrossRefGoogle Scholar
  8. 8.
    Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. Primary biliary cirrhosis. Hepatology. 2009;50(1):291–308.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Internal MedicineUniversity of Miami Miller School of Medicine/Jackson Memorial HospitalMiamiUSA
  2. 2.Division of HepatologyUniversity of Miami Miller School of MedicineMiamiUSA

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