Atsushi Tanaka1, Kenichi Harada, Hirotoshi Ebinuma, Atsumasa Komori, Junko Yokokawa, Kaname Yoshizawa, Masanori Abe, Yasuhiro Miyake, Kentaro Kikuchi, Hiromasa Ohira, Mikio Zeniya, Kazuhide Yamamoto, Hiromi Ishibashi, Morikazu Onji, Yasuni Nakanuma, Hirohito Tsubouchi, Hajime Takikawa. 1. Department of Medicine, Teikyo University School of Medicine, Tokyo Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa Department of Gastroenterology and Hepatology, Keio University School of Medicine, Tokyo Clinical Research Center, NHO Nagasaki Medical Center, Omura Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, Fukushima Department of Medicine, Division of Gastroenterology and Hepatology, Shinshu University School of Medicine, Matsumoto Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama The 4th Department of Internal Medicine, Teikyo University, School of Medicine, Kanagawa Division of Gastroenterology and Hepatology, Department of Internal Medicine, Jikei University School of Medicine, Tokyo Department of Digestive and Life-Style Related Disease, Health Research Course, Human and Environmental Sciences, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
Abstract
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 < 0.001). At the end of the follow-up period, corticosteroids were used in 23 patients (72%), and neither liver-related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92% and 75% in the training set (n = 17), and 91% and 80% in the validation set (n = 16) of overlap. Only 3% of PBC patients were diagnosed as having indication for corticosteroid use. CONCLUSION: In PBC-AIH overlap, AIH-like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.
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 < 0.001). At the end of the follow-up period, corticosteroids were used in 23 patients (72%), and neither liver-related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92% and 75% in the training set (n = 17), and 91% and 80% in the validation set (n = 16) of overlap. Only 3% of PBC patients were diagnosed as having indication for corticosteroid use. CONCLUSION: In PBC-AIH overlap, AIH-like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.