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The present study investigated the possible risk factors, including relationship/HLA matching between donor and recipient, and immunosuppressive therapies on the recurrence of primary sclerosing cholangitis (PSC) after liver transplantation (LT). Subjects were 197 recipients of LT for PSC, among whom 180 surviving more than 1 year after LT were further analyzed for risk factors of recurrence. The 5‐ and 10‐year patient‐ and graft survival rates were 83% and 68%, and 71% and 62%, respectively. The overall PSC recurrence rate was 25% with a 5‐ and 10‐year graft survival rate of 34% and 18%, which was significantly lower than the survival rate of those without recurrence (P < 0.001). Univariate analysis identified the following as risk factors for recurrence: donor age (P < 0.001), cyclosporine use (P = 0.012), mono or no immunosuppressive agent (P < 0.001), postoperative biliary complication (P < 0.001), and active intestinal bowel disease after LT (P < 0.001). Among these factors, donor age ≥45 years [hazard ratio (HR), 1.65; 95% confidence interval (CI), 1.21–2.69; P = 0.003] and mono or no immunosuppressive agent 1‐year after LT (HR, 2.38; 95% CI, 1.23–3.45; P = 0.011) were identified as independent risk factors in the final multivariate Cox regression model. The results were similar in sub‐analysis for ABO‐identical/compatible adult living donor LT cases. [ABSTRACT FROM AUTHOR]

Aim: Autoimmune liver diseases (AILD) including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) impose a significant burden on public health, and it is important to estimate their prevalence. We carried out a nationwide, hospital‐based, epidemiological survey of AIH, PBC, and PSC, and compared the results with those from previous surveys. Method: We randomly selected health‐care facilities used in the survey from a list of all facilities in Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. We sent questionnaires to the selected facilities enquiring about the number and sex of patients with AILD who visited the facility in 2016. An identical survey was undertaken for AIH/PBC in 2004 and for PSC in 2007; we carried out a comparative analysis of these data. Results: We selected 1793 departments from health centers all over Japan. Of them, 1078 (60.1%) responded to the questionnaires. The number of reported patients with AIH, PBC, and PSC was 8505, 10 847, and 906, respectively, and point prevalence was 23.9 (95% confidence interval, 23.3–24.5) for AIH (8.7 in 2004), 33.8 (33.0–34.6) for PBC (11.6 in 2004), and 1.80 (1.75–1.85) for PSC (0.95 in 2007) per 100 000 population. Male‐to‐female patient ratio of AILD was 1:4.3 for AIH (1:6.9 in 2004), 1:3.9 for PBC (1:7.1 in 2004), and 1:0.88 for PSC (1:1.4 in 2007). Conclusion: The current study indicates an increasing trend of prevalence as well as male‐to‐female patient ratio of AILD in Japan. [ABSTRACT FROM AUTHOR]

Primary sclerosing cholangitis is frequently complicated by inflammatory bowel disease. Although many colitis models have been reported, little information has been obtained about complicated cholangitis. The aim of the present study was to determine whether hepatobiliary disorders occur in mice experimental colitis, and to clarify the underlying mechanisms. The CD-1 mice were fed standard chow with or without dextran sulfate sodium in the drinking water, followed by histological examination of the liver and colon. Mononuclear cells were isolated from these organs, and cytokine production was assessed. The CD4/CD8 ratio and the population of natural killer T (NKT) cells were analyzed by flow cytometry. Inflammatory cell infiltration and focal necrosis in the liver were found in 33% of treated mice. In treated mice, the CD4/CD8 ratio increased in the liver, whereas no such change was found in the colon. Also an increase of interferon-γ and a decrease of interleukin-4 production were observed. The NKT cell population showed transient changes in the liver and colon. Hepatobiliary disorders were complicated with experimental colitis in CD-1 mice. Immunological findings indicate a T-helper-1-dominant underlying mechanism, and NKT cells may play a pathogenic role in this model. This model may help to elucidate the relationship between hepatic and colonic inflammations. © 2004 Blackwell Publishing Asia Pty Ltd [ABSTRACT FROM AUTHOR]