Cirrhosis and Autoimmune Liver Disease
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Purpose of Review
Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) constitute the most frequently observed forms of autoimmune liver diseases. Each of these autoimmune liver diseases might present with cirrhosis at diagnosis, and a significant proportion of patients develop cirrhosis during follow-up. This manuscript provides a review that addresses how to monitor and manage patients with cirrhosis secondary to autoimmune liver diseases.
For patients with PBC, the farnesoid X receptor (FXR) agonist, obeticholic acid (OCA), is the first approved drug since ursodeoxycholic acid (UDCA) and is licensed for non-responders to or those intolerant of UDCA. Bezafibrate has been shown to be effective in non-responders to UDCA, but is not licensed for this clinical indication at present. For patients with AIH, rituximab, a monoclonal antibody against the protein CD20 is a potential option for patients with suboptimal response to corticosteroids. New treatment options are currently being investigated for AIH and PSC that include the anti-B cell-activating factor receptor monoclonal antibodies, nor-UDCA, amongst others, but the efficacy in patients with cirrhosis has not been fully established.
Cirrhosis is present in 30–50% of patients at the time of diagnosis of AIH, in 20% of patients with PBC, and in 35% of patients with PSC. Therefore, cirrhosis constitutes one of the main complications of autoimmune liver disease and continues to develop at a frequency of approximately 3–6% per year. Patients with compensated AIH cirrhosis and histological inflammatory activity benefit of corticosteroid treatment. Patients with PBC and compensated cirrhosis should receive UDCA. OCA should be considered in non-responders but used with very close monitoring in patients with decompensated cirrhosis. There is no effective treatment option available for patients suffering from PSC. Liver transplantation is indicated for patients who have progressed to decompensated cirrhosis, and those with intractable symptoms or hepatocellular carcinoma within transplant criteria. Standard monitoring for cirrhosis-related complications is highly recommended.
KeywordsAutoimmune liver disease Autoimmune hepatitis Primary biliary cholangitis Primary sclerosing cholangitis Cirrhosis
Compliance with Ethical Standards
Conflict of Interest
Aldo Montano-Loza, Andrew Mason and Maryam Ebadi each declare no potential conflicts of interest. Guido Stirnimann reports personal fees from Intercept Switzerland, during the conduct of the study.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
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