Outcome and prognostic factors of 391 Japanese patients with primary sclerosing cholangitis

Objectives: We performed a national survey in 2003, and demonstrated characteristic features of primary sclerosing cholangitis (PSC) patients in Japan. In this study, we aimed to clarify the outcome and prognostic factors of Japanese PSC patients. Methods: Questionnaires were sent to gastroenterologists in Japan, and 391 patients with PSC were registered and enrolled in the current study. The median follow‐up was 5.3 years (range 0.1–20.8 years). The cumulative incidence for survival was analysed using the Kaplan–Meier method. Univariate and multivariate analyses were performed using the Cox‐proportional hazards regression model for determining prognostic variables. Results: The estimated median survival of all patients was 13.1 years, with a 5‐year survival rate of 74.5%. Thirty‐eight patients (9.7%) who underwent liver transplantation (LT) had a 5‐year survival rate of 92.0%. Both univariate and multivariate analysis demonstrated that younger age [below 49 years old; odds ratio (OR)=1.76, 1.12–2.76, P=0.0136] and lower total bilirubin (below 3.0 mg/dl; OR=2.50, 1.60–3.89, P≤0.0001) were independent prognostic factors for LT‐free survival of PSC patients in Japan. Cholangiocarcinoma (CCA) was found in 14 (3.6%) patients, and only two out of 125 PSC patients exhibited a history of inflammatory bowel diseases. Conclusions: Although several characteristic features existed, the outcome as well as prognostic factors of Japanese PSC patients appeared to be similar to those from the United States and European countries. In contrast, the incidence of CCA in PSC appeared to be lower in Japan.