By AARDA Scientific Advisory Board member M. Eric Gershwin, M.D., InFocus,Vol. 11,No.4,December,2003
Dr. Gershwin is a Distinguished Professor of Medicine, the Jack and Donald Chia Professor of Medicine, and Chief, Division of Rheumatology, Allergy and Clinical Immunology at the University of California at Davis, California.
Primary biliary cirrhosis (PBC) is an autoimmune disease of the bile ducts that predominantly affects women. In fact, it is the most female predominant of the autoimmune diseases.
The disease process of primary biliary cirrhosis often begins in an insidious fashion with unexplained itching. In fact, primary biliary cirrhosis can sometimes be diagnosed in asymptomatic patients when they are found to have an elevation of their serum alkaline phosphatase. The hallmark of this disease is the presence of autoantibodies to mitochondria in affected patients. In fact, these autoantibodies to mitochondria (coined AMA) are virtually diagnostic of primary biliary cirrhosis.
The natural history of the disease process is a very long one, and fortunately there are large numbers of patients who lead active and vigorous lives without impediment. Most patients with primary biliary cirrhosis receive treatment with an agent that chelates their bile salts and appears to reduce not only symptoms but also disease progression; this agent is called UDCA.
Our laboratory has been studying primary biliary cirrhosis for more than 15 years and has directed most of its attention on the immunology of affected patients. Since the only definitive treatment for this liver disease is liver transplantation, we hope that our research will develop new therapies. We work closely with a large group of patients on a PBC internet group. The url for this group is www.pbcers.org. To learn more about PBC, access the Web site for our laboratory (http://internalmedicine.ucdmc.ucdavis.edu/who/rheumatology/PBC_Lab/).
There are many active projects that patients can become involved in. We are, for example, identifying twins that have either one or both members affected by primary biliary cirrhosis. There is also a multi-university epidemiologic study that is being directed at the University of California at Davis and involves more than 18 other centers throughout the United States. The goals of these research projects are to identify the genetic basis of disease and also to obtain information regarding what might cause the process to begin. The process itself is a slow destruction of the small bile ducts within the liver. This causes the loss of the bile ducts and eventual obstruction of bile with resultant symptoms of itching and eventually liver failure. For more information about any of these studies, you may send an e-mail request (ksit@ucdavis.edu).
