A Case of Primary Biliary Cirrhosis in Association with Sjorgen's Syndrome Developing Features of Systemic Lupus Erythematosus / 대한류마티스학회지

ABSTRACT

Primary biliary cirrhosis (PBC)is a chronic inflammatory disease characterized by fibrous obliteration of intrahepatic bile ductules.Although the etiopathogenesis of PBC remains unclear,several studies suggest that a disordered immune response may be involved.This disease is commonly associated with various autoimmune diseases,such as Sjogren's syndrome,scleroderma, rheumatoid arthritis,Hashimoto's thyroiditis,polymyositis,and pernicious anemia.More than 80%of cases with PBC are accompanied by at least one autoimmune disorder,and 40%by two or more. Sjogren's syndrome,defined by dry eyes and xerostomia,may be found in 69 to 81%of PBC patients. However, concurrent coexistence of PBC and systemic lupus erythematosus (SLE)has been rarely described in the literature.We report a 46-year-old female with PBC and Sjogren's syndrome who also satisfied a classification criteria of SLE.