Takamura Masaaki, Matsuda Yasunobu, Kimura Naruhiro, Takatsuna Masafumi, Setsu Toru, Tsuchiya Atsunori, Osaki Akihiko, Waguri Nobuo, Yanagi Masahiko, Takahashi Toru, Sugitani Soichi, Kobayashi Yuka, Yoshikawa Akira, Ishikawa Toru, Yoshida Toshiaki, Watanabe Toshiaki, Bannai Hitoshi, Kubota Tomoyuki, Funakoshi Kazuhiro, Wakabayashi Hiroto, Kurita So, Ogata Norio, Watanabe Masashi, Mita Yuhsaku, Mori Shigeki, Sugiyama Motoya, Miyajima Toru, Takahashi Sumio, Sato Shuichi, Ishizuka Kisei, Ohta Hironobu, Aoyagi Yutaka, Terai Shuji
Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Niigata, Japan.
Department of Medical Technology, Niigata University Graduate School of Health Sciences, Niigata, Niigata, Japan.
Hepatol Res. 2021 Feb;51(2):166-175. doi: 10.1111/hepr.13586. Epub 2020 Nov 28.
Disease characteristics of primary biliary cholangitis have changed recently. However, detailed studies on the subject have been limited. Therefore, we aimed to clarify disease characteristics of patients with recent primary biliary cholangitis using the cohort from Niigata University and 21 affiliated hospitals.
Overall, 508 patients were enrolled in this study from 1982 to 2016, divided into three cohorts according to their year of diagnosis: ≤1999, 2000-2009 and ≥2010. We compared differences in clinical characteristics, response to ursodeoxycholic acid and prognosis.
The male-to-female ratio increased incrementally from 1:16.4 (≤1999) to 1:3.8 (≥2010) (P < 0.001). In women, the median age at diagnosis increased incrementally from 54.0 years (≤1999) to 60.5 years (≥2010) (P < 0.001) and serum albumin decreased gradually (P = 0.001), which might have affected the increase in the Fibrosis-4 Index and albumin-bilirubin score. The ursodeoxycholic acid response rate according to the Barcelona criteria increased incrementally from 26.7% (≤1999) to 78.4% (≥2010) (P < 0.010), and those according to other criteria (Paris-I, Rotterdam and Toronto) were approximately ≥80% in all cohorts. Ten-year survival rate in the ≤1999 and 2000-2009 cohorts were 98.6% and 95.6%, respectively. These earlier cohorts were also characterized by a higher rate of asymptomatic state and mild histology (83.5% [≤1999] and 84.7% [2000-2009], and 93.6% [≤1999] and 91.1% [2000-2009]).
Patients with primary biliary cholangitis were characterized by older age at diagnosis and an increase in male to female ratio as well as higher response rates of ursodeoxycholic acid and longer survival, resulting from the early recognition of primary biliary cholangitis.
原发性胆汁性胆管炎的疾病特征近来有所变化。然而，关于该主题的详细研究有限。因此，我们旨在利用新潟大学及其21家附属医院的队列研究来阐明近期原发性胆汁性胆管炎患者的疾病特征。
本研究共纳入了1982年至2016年间的508例患者，根据诊断年份分为三个队列：≤1999年、2000 - 2009年和≥2010年。我们比较了临床特征、对熊去氧胆酸的反应及预后方面的差异。
男女比例从1:16.4（≤1999年）逐步增至1:3.8（≥2010年）（P < 0.001）。在女性中，诊断时的中位年龄从54.0岁（≤1999年）逐步增至60.5岁（≥2010年）（P < 0.001），血清白蛋白逐渐降低（P = 0.001），这可能影响了纤维化-4指数和白蛋白-胆红素评分的升高。根据巴塞罗那标准，熊去氧胆酸的反应率从26.7%（≤1999年）逐步增至78.4%（≥2010年）（P < 0.010），而根据其他标准（巴黎-I、鹿特丹和多伦多），所有队列中的反应率均约≥80%。≤1999年和2000 - 2009年队列的10年生存率分别为98.6%和95.6%。这些较早队列的特点还包括无症状状态和轻度组织学的比例较高（≤1999年为83.5%，2000 - 2009年为84.7%；≤1999年为93.6%，2000 - 2009年为91.1%）。
原发性胆汁性胆管炎患者的特征为诊断时年龄较大、男女比例增加、熊去氧胆酸反应率较高及生存期延长，这是由于对原发性胆汁性胆管炎的早期识别所致。
