Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome: A rationale for corticosteroids use based on a nation-wide retrospective study in Japan

• Published in: Hepatology research 2011-09, Vol.41 (9), p.877-886
• Main Authors: Tanaka, Atsushi, Harada, Kenichi, Ebinuma, Hirotoshi, Komori, Atsumasa, Yokokawa, Junko, Yoshizawa, Kaname, Abe, Masanori, Miyake, Yasuhiro, Kikuchi, Kentaro, Ohira, Hiromasa, Zeniya, Mikio, Yamamoto, Kazuhide, Ishibashi, Hiromi, Onji, Morikazu, Nakanuma, Yasuni, Tsubouchi, Hirohito, Takikawa, Hajime
• Format: Article
• Language: English
• Subjects: Cholangitis; Corticoids; corticosteroids; Hepatitis; Liver diseases; Liver transplantation; primary biliary cirrhosis; the revised International Autoimmune Hepatitis Group scoring system; the simplified scoring system; ursodeoxycholic acid
• ISSN: 1386-6346; 1872-034X
• DOI: 10.1111/j.1872-034X.2011.00844.x

Aims:  Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC‐AIH overlap syndrome. Previous studies suggest that combination therapy of ursodeoxycholic acid (UDCA) and corticosteroids may be effective. In the current study, we aimed to describe clinical features of these cases and to propose a rationale for combination treatment in PBC‐AIH overlap. Methods:  We enrolled patients with PBC‐AIH overlap from eight referral centers for liver diseases in Japan, and clinical, biochemical and immunological features were examined. Liver histology of all patients at diagnosis were analyzed altogether in detail. Eighty‐nine and 44 patients with PBC and AIH alone were included and served as controls. Results:  We identified 33 patients with PBC‐AIH overlap. The mean follow‐up period was 6.1 years. On liver histology, the HA (hepatitis activity) score was significantly higher than the CA (cholangitis activity) score (P