A case of cholestatic autoimmune hepatitis and acute liver failure: an unusual hepatic manifestation of mixed connective tissue disease and Sjogren'ssyndrome

Although hepatomegaly is reported to occur occasionally in patients with mi xed connective tissue disease (MCTD) or Sjogren's syndrome (SS), autoimmune liver diseases such as primary biliary cirrhosis, sclerosing cholangitis, and autoimmune hepatitis in association with MCTD or SS have rarely been de scribed. We report a case of severe cholestatic autoimmune hepatitis presen ting with acute liver failure in a 40-yr-old female patient suffering from MCTD and SS. The diagnosis of MCTD and SS was made at the age of 38. The pa tient presented severe jaundice and elevation of conjugated bilirubin. The patient denied alcohol and drug use and had no evidence of viral hepatitis. On the 8th, day of her hospitalization, the patient developed grade III he patic encephalopathy. She was diagnosed as autoimmune hepatitis presenting with acute liver failure based on clinical features, positive FANA and anti -smooth muscle antibodies, negative anti-mitochondrial antibodies, high tit ers of serum globulin, liver biopsy findings, and a good response to cortic osteroid therapy, The patient was managed with prednisolone and the clinica l symptoms, liver function test results, and liver biopsy findings showed m uch improvement after steroid therapy.