Primary Biliary Cholangitis and Autoimmune Hepatitis - 09/05/18

Doi : 10.1016/j.path.2018.02.010

Raul S. Gonzalez, MD ^a,^⁎ , Kay Washington, MD, PhD ^b
^a Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, 601 Elmwood Avenue, Box 626, Rochester, NY 14642, USA
^b Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, 1161 21st Avenue South, C-3316 MCN, Nashville, TN 37232-2561, USA

^∗Corresponding author.

Abstract

Primary biliary cholangitis and autoimmune hepatitis are common autoimmune diseases of the liver. Both have typical clinical presentations, including certain autoantibodies on serologic testing. Histologic features are also often typical: primary biliary cholangitis shows bile duct destruction (sometimes with granulomas), and autoimmune hepatitis shows prominent portal and lobular lymphoplasmacytic inflammation. Both have a wide differential diagnosis, including one another; they may also simultaneously occur within the same patient. Careful use of clinical and histologic criteria may be necessary for diagnosis. First-line therapy is immunosuppression for autoimmune hepatitis and ursodeoxycholic acid for primary biliary cholangitis. Both diseases may progress to cirrhosis.

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Keywords : Primary biliary cholangitis, Primary biliary cirrhosis, Autoimmune hepatitis, Biliary disease, Chronic hepatitis, Overlap syndrome, Autoantibodies, Differential diagnosis