Cholestatic liver diseases encompass a broad spectrum of pathologies, with the core injury occurring at the level of cholangiocytes and progressing to hepatic fibrosis and liver dysfunction. Primary biliary cholangitis and primary sclerosing cholangitis are the most significant progressive cholangiopathies in adults. Although rare, they commonly evolve to liver failure and need for liver transplantation. Despite recent advances in the basic knowledge of these cholangiopathies, the pathogenesis is still elusive. Targeted treatments to prevent disease progression and to preclude malignancy are not yet available. This review will address the general clinical features of both diseases, analyze their commonalities and differences, and provide a state-of-the art overview of the currently available therapeutics.
1Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic, Scottsdale, Arizona, USA;
2Transplant Hepatology Fellow, Mayo Clinic School of Graduate Medical Education, Scottsdale, Arizona, USA;
3Transplant Center, Mayo Clinic, Phoenix, Arizona, USA.
Correspondence: Elizabeth J. Carey, MD. E-mail: Carey.Elizabeth@mayo.edu.
SUPPLEMENTARY MATERIAL accompanies this paper at http://links.lww.com/AJG/A181
Received April 10, 2019
Accepted April 10, 2019
Online date: June 4, 2019