Coexistence of Primary Biliary Cirrhosis and Immune Thrombocytopenic Purpura

Although autoimmune diseases are often concomitant, the coexistence of primary biliary cirrhosis (PBC) and immune (idiopathic) thrombocytopenic purpura (ITP) is rare. This is a review of the English-language literature regarding concomitant cases of PBC and ITP. Among 17 concomitant cases reported, including four diagnosed with Evans syndrome, which includes ITP symptoms, PBC was diagnosed first in five cases, ITP was diagnosed first in two cases, and both were almost simultaneously diagnosed in remaining 10 cases. Standard pharmacotherapy was ursodeoxycholic acid (UDCA) for PBC and corticosteroids for ITP/Evans syndrome. Among these 17 cases, one fatality was observed although there appears to be no trend for worse outcome compared with either PBC or ITP alone.