Background and Aims

Recent studies point out to epidemiological changes in primary sclerosing cholangitis (PSC). Our aims were to determine in PSC patients followed in several centers in a Mediterranean geographic area: (i) changes in baseline features and (ii) effect of gender on clinical course.

Methods

Retrospective multicenter study of PSC patients treated in 8 hospitals in a Mediterranean area between 2000 and 2021. Charts were reviewed compiling demographic, clinical, radiological, and histological variables.

Results

Cohort of 112 PSC patients included, 42% women, 70% diagnosed after 2010. Women were increasingly diagnosed in recent cohorts. The median time from diagnosis to the combined endpoint liver transplantation (Lt) and/or death was 6.9 years. Asthenia at diagnosis (p = 0.009) was associated with lower transplant-free survival, while diagnosis before 2005 was associated with greater LT-free survival (p < 0.001). By Cox regression, LT-free survival was not influenced by age, sex, or cirrhosis at the time of diagnosis. Women were found to have less jaundice at diagnosis (2 vs 14%; p = 0.013), higher prevalence of ANA antibodies (43.9 vs 15.7%; p = 0.003), and lower GGT levels at diagnosis (GGT 123 vs 209U/L; p = 0.014) than men.

Conclusion

In an area traditionally considered to have low prevalence, the prevalence of affected women surpasses expectations based on existing literature. There appear to be gender-related variations in the presentation of the condition, highlighting the need for confirmation through larger-scale studies.

中文翻译：

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原发性硬化性胆管炎：巴伦西亚低发病率地区的性别影响

背景和目标

最近的研究指出了原发性硬化性胆管炎（PSC）的流行病学变化。我们的目的是确定在地中海地理区域的几个中心随访的 PSC 患者：(i) 基线特征的变化和 (ii) 性别对临床病程的影响。

方法

对 2000 年至 2021 年间在地中海地区 8 家医院接受治疗的 PSC 患者进行的回顾性多中心研究。对图表进行了审查，汇编了人口统计学、临床、放射学和组织学变量。

结果

由 112 名 PSC 患者组成的队列包括 42% 的女性，70% 是在 2010 年之后诊断的。在最近的队列中，女性被诊断的人数越来越多。从诊断到联合终点肝移植 (Lt) 和/或死亡的中位时间为 6.9 年。诊断时的无力 ( p  = 0.009) 与较低的无移植生存率相关，而 2005 年之前的诊断则与较高的无移植生存率相关 ( p  < 0.001)。根据 Cox 回归，无 LT 生存期不受诊断时年龄、性别或肝硬化的影响。研究发现，女性在诊断时黄疸较少（2% vs 14%；p  = 0.013），ANA 抗体患病率较高（43.9% vs 15.7%；p  = 0.003），并且诊断时 GGT 水平较低（GGT 123 vs 209U/L；p = 0.003）。p  = 0.014）高于男性。

结论

在传统上被认为患病率较低的地区，受影响妇女的患病率超出了现有文献的预期。这种情况的表现似乎存在与性别相关的差异，强调需要通过更大规模的研究来确认。