Department of Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, PO Box 30001, 9700 RB Groningen, The Netherlands.
Dig Liver Dis. 2011 Oct;43(10):802-6. doi: 10.1016/j.dld.2011.05.009. Epub 2011 Jun 22.
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.
To assess the prevalence of concomitant immune-mediated diseases and the impact on disease outcome in PSC.
We included 241 patients and retrospectively reviewed medical charts.
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.
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.
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。
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。
我们纳入了 241 名患者，并回顾性地审查了病历。
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。
在大型 PSC 人群中，发现同时存在多种免疫介导性疾病的患病率较高。IBD 更多地发生在早期获得性 PSC 中，而其他免疫介导性疾病更多地发生在晚期获得性 PSC 中。非 IBD 免疫介导性疾病对结局无影响。
