Primary Biliary Cirrhosis

In this study, we aim to identify gut microbiomes specific to patients with chronic refractory liver disease and to conduct a gut-liver axis study on the pathogenesis and disease progression.

This study is a biobank of specimens and clinical data for use in current and future research to better understand the cholestatic liver diseases primary biliary cirrhosis/cholangitis (PBC) and primary sclerosing cholangitis (PSC).

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access. Visit sanfordresearch.org/CoRDS to enroll.

Up to 40% of patients with PBC have an inadequate response to standard treatment with UDCA, hence bezafibrate, a PPAR-agonist is being introduced as add-on therapy in these patients. sCD163, fibrosis markers and bile acid composition are of special interest in PBC. In this study, the investigators will investigate how treatment with bezafibrate influence levels of macrophage activation markers and fibrosis markers as well as bile acid composition in patients offered bezafibrate as add-on therapy to UDCA.

Primary biliary cholangitis (PBC) is an autoimmune chronic liver disease, characterised by destruction of the small intrahepatic bile ducts. Ursodeoxycholic acid (UDCA) is the first line treatment for patients with PBC. However, up to 40% of patients respond inadequate to this treatment. sCD163 is a macrophage activation marker shedded into plasma by macrophages in the liver. sMR is a soluble mannose receptor. The investigators want to investigate whether sCD163 and sMR can predict response to treatment with UDCA in newly diagnosed patients with PBC.