Primary biliary cirrhosis and systemic lupus erythematosus. A rare association.

Although the co-existence between primary biliary cirrhosis (PBC) and one or more autoimmune diseases is very common, the association with systemic lupus erythematosus (SLE) is believed to be rare. We describe a 37-year-old woman with PBC who developed clinical and serological features of SLE 4 years later. The anti-mitochondrial antibody (AMA) titers fell to undetectable levels during the acute phase of the SLE. At the same time, high titers of antinuclear antibodies (ANA) were detected. Changes in opposite directions in the AMA and ANA titers were also seen during remission of the SLE.