Primary biliary cirrhosis: a Mount Sinai perspective.

Individuals afflicted with primary biliary cirrhosis (PBC) first undergo chronic, nonsuppurative destruction of their intrahepatic bile ducts, eventually leading to cirrhosis. Over nearly 50 years, many faculty members at the Mount Sinai School of Medicine, including Dr. Hans Popper and Dr. Fenton Schaffner, have made important contributions to our understanding of the natural history and histopathologic evolution of PBC. And today, many patients with PBC continue to be cared for at Mount Sinai. In the absence of a cure for the disease, these patients continue to be enrolled in clinical trials and, when necessary, in the Mount Sinai liver transplant program. The establishment of the Center for the Study of Primary Biliary Cirrhosis at Mount Sinai, supported by the Artzt Family Foundation Trust, has enabled the faculty to expand both clinical and basic science initiatives related to primary biliary cirrhosis. Several of these new initiatives are described below and placed in the context of our current understanding of the immunopathogenesis of PBC.