J Korean Med Sci.  2001 Aug;16(4):512-515. 10.3346/jkms.2001.16.4.512.

A Case of Cholestatic Autoimmune Hepatitis and Acute Liver Failure: an Unusual Hepatic Manifestation of Mixed Connective Tissue Disease and Sjogren's Syndrome

• Min JK
• Han NI
• Kim JA
• Lee YS
• Cho CS
• Kim HY

• KMID: 648313
• DOI: http://doi.org/10.3346/jkms.2001.16.4.512

Abstract

Although hepatomegaly is reported to occur occasionally in patients with mixed 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 described. We report a case of severe cholestatic autoimmune hepatitis presenting 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 patient 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 hepatic 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 titers of serum globulin, liver biopsy findings, and a good response to corticosteroid therapy, The patient was managed with prednisolone and the clinical symptoms, liver function test results, and liver biopsy findings showed much improvement after steroid therapy.

Keyword

Hepatitis; Mixed Connective Tssue Disease; Sjogren's Syndrome

MeSH Terms

Adult
Cholestasis/*etiology
Female
Hepatitis, Autoimmune/*etiology
Human
Liver Failure, Acute/*etiology
Mixed Connective Tissue Disease/*complications
Sjogren's Syndrome/*complications