Mixed types of chronic active hepatitis and primary biliary cirrhosis associated with the anti-phospholipid antibody syndrome: a case report.

An understanding of the pathogenesis of mixed types of chronic active hepatitis and primary biliary cirrhosis is important for the treatment of the patients. A 40-year-old Japanese woman with antiphospholipid syndrome has been treated with prednisolone for two years since she was diagnosed as having a mixed type of chronic active hepatitis and primary biliary cirrhosis. Biochemical tests for liver function were normal during treatment. Laparoscopy revealed a white liver, and histology demonstrated disappearance of the findings of piecemeal necrosis or chronic nonsuppurative destructive cholangitis in the specimen. Steroid treatment of patients with mixed types of chronic active hepatitis and primary biliary cirrhosis is controversial, since it is contra-indicated in some. Although the clinical features of the patients varies from one to another, this case suggests that the autoimmune mechanism of some patients may resemble autoimmune hepatitis rather than primary biliary cirrhosis, and treatment with steroid is effective.