[A case of Sjögren's syndrome associated with advanced hemolytic anemia caused by worsening autoimmune cholangiopathy].

We report a rate case of autoimmune cholangiopathy (AIC) and autoimmune hemolytic anemia (AIHA) in a patient with Sjögren's syndrome. A 59-year-old woman was admitted to Matsuyama Red Cross Hospital in September 1996 because of worsening liver dysfunction. She had suffered from keratoconjunctivitis sicca and xerostomia and had been diagnosed as having Sjögren's syndrome in February 1994, based on histological examination of the minor salivary gland and sialography, and positivity for SS-A and SS-B antibody. Liver dysfunction had first become evident in September 1995. Histological examination of a liver biopsy specimen obtained by laparoscopy showed that the structure of the hepatic lobules was mostly preserved, whereas most of the biliary ducts were transformed, being consistent with AIC. On admission, the patient was given 40 mg of prednisolone. Although the serum transaminase level decreased, jaundice persisted and hemolytic anemia developed. Further administration of 60 mg of prednisolone and plasmapheresis ameliorated the hemolytic anemia and cured the jaundice. We consider that an increased immunological response caused by the worsening AIC might have played a role in the development of AIHA in the present case.