Antimitochondrial antibody-negative primary biliary cirrhosis in an elderly African American man

Primary biliary cirrhosis (PBC) is a chronic, progressive autoimmune liver disease, which is characterized by nonsuppurative destruction of intrahepatic ducts, resulting in cholestasis, cirrhosis, and end-stage liver disease. Antimitochondrial antibodies (AMAs) are considered to be a highly sensitive and specific serological marker of PBC; however, a subset of patients who have biochemical and histopathological features consistent with PBC do not have detectable AMAs in their serum. This phenomenon is referred to as AMA-negative PBC. This article briefly reviews the literature on AMA-negative PBC and discusses the diagnosis and treatment of this condition in older adults.