Clinical features of hepatocellular carcinoma in patients with autoimmune hepatitis in Japan.

Background: The occurrence of hepatocellular carcinoma (HCC) in patients with autoimmune hepatitis (AIH) is rare compared to that in patients with viral hepatitis. To clarify the status of HCC in patients with AIH in Japan, the clinical features of HCC in patients with AIH were analyzed.
Methods: A primary survey gathered data from 496 member institutions of the Liver Cancer Study Group of Japan, and a secondary survey collected additional information from 250 HCC patients out of a total 4869 AIH patients who were identified in the primary survey.
Results: Of the 250 patients identified through the secondary survey, 127 patients (50.8 %) from throughout Japan were found to have HCC. The mean age at diagnosis of HCC was 69 years, and the male-to-female ratio was 1:5.7. The mean period from diagnosis of AIH to detection of HCC was 8 years, and Child-Pugh status at the time of HCC diagnosis was class A in 61.8 %; of the patients analyzed, 77.9 % also had cirrhosis of the liver. The mean value of maximum tumor diameter was 4.3 cm, and clinical stages were I in 20.1 % of patients, II in 47.6 %, III in 23.4 %, and IV in 8.9 %. The therapeutic modality used was surgery in 30.2 %, percutaneous therapy in 29.5 %, transcatheter arterial chemoembolization in 36.4 %. Cumulative survival rate was 85.4 % at one year.
Conclusion: The survey results showed that HCC developed in 5.1 % of patients with AIH in Japan, with cirrhosis of the liver commonly found in elderly individuals; when HCC was diagnosed at an early clinical stage, in many cases, the liver function was relatively preserved. After diagnosis of AIH, observation of its progression with close attention to potential HCC complications is necessary.