Abstract
Primary Biliary Cirrhosis (PBC) is the most common chronic cholestatic liver disease in adults in the United States (16). There are several rare conditions associated with PBC. We review all reported associations and present our own case experience. The list of these conditions is extensive but the numbers of cases are limited. Our aim is to make the physicians aware of these rare associations and help in the diagnosis. A few cases of multiple myeloma in early stages of PBC have been described (3). We report a patient who presented with severe coagulopathy in whom antimitochondrial antibodies were positive. Despite normal liver transaminases and alkaline phosphatase as in previously reported cases (13), liver biopsy showed advanced cirrhosis and histological changes consistent with PBC. A concurrent diagnosis of multiple myeloma IGG type was made by bone marrow biopsy. A possible pathophysiologic mechanism is described.
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