患有与CREST综合征复杂的原发性胆道胆管炎的患者的长期结果
Kazumichi Abe1, Manabu Hayashi2, Tatsuro Sugaya2
1Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. k-abe@fmu.ac.jp.
Scientific reports
|June 19, 2024
概括
患有原发性胆道胆炎和CREST综合征 (PBC-CREST) 的患者与单独患有原发性胆道胆炎 (PBC) 的患者相比,显示出更好的长期结果和生存率. 在PBC患者中,CREST综合征似乎是预防肝硬化的一个保护因素.
科学领域:
- 肝病学 肝病学是一种肝病学.
- 自免疫性疾病 自免疫性疾病
- 临床结果研究研究
背景情况:
- 初级胆道胆炎 (PBC) 是一种自身免疫性肝脏疾病,通常与其他自身免疫性疾病同时发生.
- 虽然认可了与CREST综合征 (PBC-CREST) 复杂的PBC,但其长期预后仍未得到充分研究.
- 了解PBC-CREST的结果对于患者管理和风险分层至关重要.
研究的目的:
- 为了评估PBC-CREST患者的长期结果.
- 为了验证和比较单独PBC和PBC-CREST队列之间的GLOBE和UK-PBC风险得分.
- 确定影响PBC患者CREST综合征和没有CREST综合征的预后的因素.
主要方法:
- 在1990年至2021年期间,对302名被诊断患有PBC的患者进行了回顾性分析.
- 仅PBC (n=245) 和PBC-CREST (n=57) 组之间的无肝移植 (LT) 存活率的比较.
- 在子组中验证和比较GLOBE和UK-PBC得分,但不包括早期肝脏相关事件/LT.
主要成果:
- 与单独使用PBC (92/87/80%) (P=0.0172) 相比,PBC-CREST患者在3,5年和10年表现出明显更好的无LT生存率 (98/96/96%) (P=0.0172).
- 在多变量分析中,CREST综合征被确定为抗肝硬化发展的独立保护因素.
- 在PBC-CREST组中,UK-PBC和GLOBE评分预测了与肝脏相关的死亡/LT风险显著降低,而在PBC-CREST组中,无LT生存率更高.
结论:
- 患有PBC-CREST的患者可能比单独患有PBC的患者有更好的长期结果.
- 存在CREST综合征与PBC中更有利的预后有关.
- 像GLOBE和UK-PBC这样的风险分层分数需要在PBC-CREST患者中仔细考虑,因为风险概况可能不同.
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