Improvement of IgG4-associated autoimmune cholangitis in a patient with HCV-cirrhosis by treatment with sofosbuvir and ledipasvir: a case report

Background: Hepatitis C virus (HCV) causes not only liver disease that may contribute to cirrhosis and liver failure but also can cause extrahepatic manifestations in nearly 75% ranging from cryoglobilinaemia to lymphoma. As a new clinical entity IgG4-associated autoimmune cholangitis (AIC) has gained attention characterized by elevated serum IgG4-level and diffuse cholangiographic abnomalities that respond well to steroid therapy.

Case report: A 85-year old female patient with IgG4-associated AIC and HCV GT 1b cirrhosis was sucessfully treated with sofosbuvir (SOF) and ledipasvir (LED). Viral load was below detection after 4 weeks of treatment. SVR 24 has been achieved. Intriguingly, IgG4-levels decreased by the antiviral treatment from over 1'2750 mg/l to 5728,6 mg/l within 4 month. Patient characteristics, data on lab results, and the course of disease and treatment were documented from November 2014 to September 2015.

Conclusion: This is the first reported case of IgG4-related AIC improving by antiviral treatment of HCV. Even if the pathogenetic factors of IgG4-associated AIC remains not fully understood, these finding support the assumption that inflammatory processes, in this case the chronic active HCV infection, implicated the IgG4-related disease and antiviral treatment leads to remission.