Pathogenetic and therapeutic aspects of chronic cholestatic and immune-mediated liver diseases

The pathophysiology of chronic cholestatic and immune-mediated liver diseases like primary biliary cholangitis, primary sclerosing cholangitis, autoimmune hepatitis, IgG4-related cholangitis and various genetic cholestatic disorders is still only partly understood. Patients may suffer from fatigue, pruritus and various other symptoms. Most of these diseases have a progressive course without adequate treatment leading to cirrosis and complications of portal hypertension. Evidence-based therapies are now available for some of these diseases such as primary biliary cholangitis, autoimmune hepatitis or IgG4-related disease, but many more efforts are needed to disclose the pathophysiological mechanisms leading to these diseases as well as the symptoms like pruritus and fatique. Using the full spectrum of laboratory techniques, ranging from basic biochemistry and in vitro cell biology to mouse models, translational studies and clinical trials with affected patients, we try to gain more insight in the pathophysiology of, and possible novel therapeutic approaches towards cholestatic and immune-mediated liver diseases.