Hepatology research : the official journal of the Japan Society of Hepatology · 2025
Clinical Characteristics and Long-Term Prognosis of Primary Biliary Cholangitis in Japan: Results of the 2nd-Generation Nationwide Survey.
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Kugiyama Yuki, Abe Masanori, Namisaki Tadashi, Yoshiji Hitoshi, Abe Kazumichi, Ohira Hiromasa, Shirahashi Ryosaku, Yokoyama Keiji, Fukunaga Atsushi, Kawata Kazuhito, Umemura Masahiro, Honda Akira, Ikegami Tadashi, Miyase Shiho, Nakano Toshiaki, Komori Atsumasa, Tanaka Atsushi, Japan PBC Study Group
Аннотация
AIM: The Japan Primary Biliary Cholangitis Study Group (JPBCSG) has conducted nationwide surveys of primary biliary cholangitis (PBC) since 1980. Due to the progress in the management of PBC, a more comprehensive survey platform is desired.
METHODS: The JPBCSG conducted the 17th survey of PBC by using an electronic data capture (EDC) system: The patients' clinical demography at diagnosis and the outcomes of newly and past registered cases (till the 16th) were entered jointly in the EDC system. A questionnaire for treatment response was included in the survey. Using the new platform, the trends in clinical demography and the long-term prognosis of PBC were analyzed.
RESULTS: The percentage of male patients with PBC showed a significant increase from the period before the end of 2010 [Period 1, 12.4% (n = 772)] to the period after 2021 [Period 4, 20.6% (n = 345), p < 0.001] (n = 2579, in total). The mean age at diagnosis also rose significantly from 56.8 years old in Period 1 to 61.8 years in Period 4 (p < 0.001). There were no significant changes in the prevalence of symptoms at diagnosis. In a multivariate Cox-regression analysis, older age (hazard ratio [HR] 3.48, p = 0.001), female sex (HR 0.46, p = 0.046), total bilirubin (HR 3.84, p = 0.009), fluid retention (HR 5.69, p < 0.001), and esophagogastric varices (HR 2.66, p = 0.015) were significantly associated with liver-related mortality and liver transplantation.
CONCLUSION: We have established the 2nd-generation nationwide survey of PBC in Japan. The survey results revealed not only demographical trends in Japanese patients with PBC but also risk factors for their prognosis.
METHODS: The JPBCSG conducted the 17th survey of PBC by using an electronic data capture (EDC) system: The patients' clinical demography at diagnosis and the outcomes of newly and past registered cases (till the 16th) were entered jointly in the EDC system. A questionnaire for treatment response was included in the survey. Using the new platform, the trends in clinical demography and the long-term prognosis of PBC were analyzed.
RESULTS: The percentage of male patients with PBC showed a significant increase from the period before the end of 2010 [Period 1, 12.4% (n = 772)] to the period after 2021 [Period 4, 20.6% (n = 345), p < 0.001] (n = 2579, in total). The mean age at diagnosis also rose significantly from 56.8 years old in Period 1 to 61.8 years in Period 4 (p < 0.001). There were no significant changes in the prevalence of symptoms at diagnosis. In a multivariate Cox-regression analysis, older age (hazard ratio [HR] 3.48, p = 0.001), female sex (HR 0.46, p = 0.046), total bilirubin (HR 3.84, p = 0.009), fluid retention (HR 5.69, p < 0.001), and esophagogastric varices (HR 2.66, p = 0.015) were significantly associated with liver-related mortality and liver transplantation.
CONCLUSION: We have established the 2nd-generation nationwide survey of PBC in Japan. The survey results revealed not only demographical trends in Japanese patients with PBC but also risk factors for their prognosis.