原发性硬化性胆管炎的免疫介导性疾病。

Immune-mediated diseases in primary sclerosing cholangitis.

机构信息

Department of Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, PO Box 30001, 9700 RB Groningen, The Netherlands.

出版信息

Dig Liver Dis. 2011 Oct;43(10):802-6. doi: 10.1016/j.dld.2011.05.009. Epub 2011 Jun 22.

DOI:10.1016/j.dld.2011.05.009

PMID:21700515

Abstract

BACKGROUND

Primary sclerosing cholangitis is a chronic cholestatic liver disease. An immune aetiology is suggested by associations between PSC and inflammatory bowel disease. Data on concomitant prevalence of other immune-mediated diseases is limited.

AIM

To assess the prevalence of concomitant immune-mediated diseases and the impact on disease outcome in PSC.

METHODS

We included 241 patients and retrospectively reviewed medical charts.

RESULTS

Altogether 172 (71.4%) patients had concomitant immune-mediated disease, including IBD (149, 61.8%), autoimmune hepatitis (15, 6.2%) and other immune-mediated diseases (47, 19.5%). Thirty nine patients (22.7%) had more than one immune-mediated disease other than PSC. Most frequent extrahepatic non-IBD immune-mediated diseases were sarcoidosis, thyroid disease, and type I diabetes mellitus. Age at PSC diagnosis was lower in patients with IBD. In patients with other immune-mediated diseases than autoimmune hepatitis or IBD, age at PSC diagnosis was higher. Younger age at diagnosis and concomitant IBD related to longer survival till death or liver transplantation.

CONCLUSIONS

In a large PSC population, a high prevalence of concomitant immune-mediated diseases was found. IBD occurred more often in early-acquired PSC, and the other immune-mediated diseases more often in later-acquired PSC. No effect on outcome was found for non-IBD immune mediated disease.

摘要

背景

原发性硬化性胆管炎是一种慢性胆汁淤积性肝病。PSC 与炎症性肠病之间的关联提示其病因与免疫相关。关于其他免疫介导性疾病同时发生的患病率的数据有限。

目的

评估 PSC 中同时发生的免疫介导性疾病的患病率及其对疾病结局的影响。

方法

我们纳入了 241 名患者，并回顾性地审查了病历。

结果

共有 172 名（71.4%）患者同时患有免疫介导性疾病，包括 IBD（149 例，61.8%）、自身免疫性肝炎（15 例，6.2%）和其他免疫介导性疾病（47 例，19.5%）。39 名患者（22.7%）除 PSC 外还有一种以上的免疫介导性疾病。最常见的肝外非 IBD 免疫介导性疾病是结节病、甲状腺疾病和 1 型糖尿病。IBD 相关 PSC 患者的 PSC 诊断年龄较低。在患有除自身免疫性肝炎或 IBD 以外的其他免疫介导性疾病的患者中，PSC 的诊断年龄较高。诊断年龄较小和同时患有 IBD 与直至死亡或肝移植的生存时间延长相关。