Autoimmune Liver Diseases

EXTRAHEPATIC AUTOIMMUNE (EHA) DISEASES ASSOCIATED WITH PRIMARY BILIARY CHOLANGITIS (PBC)

Kidist K.Yimam, MD Medical Director, Autoimmune Liver Disease Program Division of Hepatology and Liver Transplantation California Pacific Medical Center (CPMC) San Francisco, CA U.S.A. PBCers 2018 Conference

OUTLINE

Brief overview Epidemiology and natural history of PBC associated extrahepatic

autoimmune (EHA) diseases In patients with PBC PBC compared with matched controls Autoimmune diseases in relatives of patients with PBC Impact of EHA conditions on PBC patients' survival Impact of response to ursodeoxycholic acid (UDCA) on EHA diseases AIH-PBC overlap syndrome and EHA disease Take home points

PRIMARY BILIARY CHOLANGITIS (PBC)

PBC is characterized by a Tlymphocyte-mediated attack on small intrahepatic bile ducts

Leads to gradual destruction and eventual disappearance of the small bile ducts (ductopenia)

florid bile duct lesion of PBC

Causes the signs and symptoms of cholestasis (such as itching) and overtime, leads to cirrhosis and liver failure

Kaplan MM. Primary biliary cirrhosis. N Engl J Med. 1996;335(21):1570.

DIAGNOSTIC APPROACH

PBC should be suspected in patients with persistent cholestatic abnormalities in serum liver tests or symptoms including pruritus or fatigue

An abnormal serum level of alkaline phosphatase (ALP) is typical in patients with PBC, mainly affecting women (female>male,10:1)

Careful personal, social, travel and family history taking may provide critical clues for the diagnosis of a cholestatic liver disease of unknown origin, particularly presence of other autoimmune diseases

Kaplan MM. Primary biliary cirrhosis. N Engl J Med. 1996;335(21):1570.

CLINICAL FEATURES

PBC vs. Primary Sclerosing Cholangitis (PSC)

Autoimmunity

T. H. Karlsen; M. Vesterhus; K. M. Boberg. Controversies in the Management of Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis. Aliment Pharmacol Ther. 2014;39(3):282-30

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