Two cases of primary biliary cirrhosis associated with systemic lupus erythematosus

Abstract

The first case is a 65-year-old woman who was diagnosed in 1987 with SLE on the basis of malar rash, photosensitivity, high ANA titer and positive anti-DNA antibody. Treatment with prednisolone resulted in improvement of her symptoms. She was followed and remained in good condition with prednisolone (10 mg/every other day). In 2006, she was admitted to our hospital for examination of abnormal liver function tests. PBC was disclosed by elevated levels of ALP and γ-GTP, positive antimitochondrial M2 antibody and liver histology (compatible with stage 2 PBC). The second case is a 55-year-old woman who was suspected in 2003 of having PBC due to elevated biliary liver enzymes and positive antimitochondrial antibody. A liver biopsy was not performed. Ursodeoxycholic acid therapy was started and was effective. In 2006, she was admitted to our hospital due to complaints of arthralgia and fatigue. Abdominal ultrasonography disclosed ascites. She was diagnosed with SLE on the basis of exudative ascites, arthritis, and positive anti-DNA and anti-Sm antibodies. Liver biopsy showed fibrous enlargement and lymphocytic infiltration of the portal areas. She was started on medication with 20 mg of prednisolone daily, and her arthritis and exudative ascites improved. PBC complicated with SLE is rare, and we report two cases with a brief discussion based on the literature. © 2011 The Japan Society of Hepatology.