Division of GI Services, University College London Hospitals NHS Foundation Trust, London, UK.
Eur J Gastroenterol Hepatol. 2012 Sep;24(9):1051-8. doi: 10.1097/MEG.0b013e3283554bbf.
Dominant biliary strictures occur commonly in patients with primary sclerosing cholangitis (PSC), who have a high risk of developing cholangiocarcinoma (CC). The natural history and optimal management of dominant strictures remain unclear, with some reports suggesting that endoscopic interventions improve outcome.
We describe a 25-year experience in patients with PSC-related dominant strictures at a single tertiary referral centre.
A total of 128 patients with PSC (64% men, mean age at referral 49 years) were followed for a mean of 9.8 years. Eighty patients (62.5%) with dominant biliary strictures had a median of 3 (range 0-34) interventions, compared with 0 (0-7) in the 48 patients without dominant strictures (P<0.001). Endoscopic interventions included the following: (i) stenting alone (46%), (ii) dilatation alone (20%), (iii) dilatation and stenting (17%) and (iv) none or failed intervention (17%, of whom most required percutaneous transhepatic drainage). The major complication rate for endoscopic retrograde cholangiopancreatography was low (1%). The mean survival of those with dominant strictures (13.7 years) was worse than that for those without dominant strictures (23 years), with much of the survival difference related to a 26% risk of CC developing only in those with dominant strictures. Half of those with CC presented within 4 months of the diagnosis of PSC, highlighting the importance of a thorough evaluation of new dominant strictures.
Repeated endoscopic therapy in PSC patients is safe, but the prognosis remains worse in the subgroup with dominant strictures. In our series, dominant strictures were associated with a high risk of developing CC.
原发性硬化性胆管炎（PSC）患者常发生优势胆管狭窄，此类患者发生胆管癌（CC）的风险较高。优势狭窄的自然病程和最佳治疗方法仍不清楚，一些报道表明内镜介入可改善预后。
我们描述了单中心 25 年治疗 PSC 相关优势狭窄的经验。
共有 128 例 PSC 患者（64%为男性，就诊时平均年龄为 49 岁）接受了平均 9.8 年的随访。80 例（62.5%）有优势胆管狭窄的患者中位干预次数为 3 次（范围 0-34 次），而 48 例无优势狭窄的患者为 0（0-7 次）（P<0.001）。内镜介入治疗包括以下几种：（i）单纯支架置入（46%），（ii）单纯扩张（20%），（iii）扩张联合支架置入（17%）和（iv）无干预或失败（17%，其中大多数需要经皮经肝引流）。内镜逆行胰胆管造影的主要并发症发生率较低（1%）。有优势狭窄的患者的平均生存时间（13.7 年）差于无优势狭窄的患者（23 年），大部分生存差异与仅在有优势狭窄的患者中发生的 26%的 CC 发病风险相关。有一半的 CC 患者在诊断 PSC 后 4 个月内出现，这突显了对新出现的优势狭窄进行彻底评估的重要性。
PSC 患者重复内镜治疗是安全的，但在有优势狭窄的亚组中预后仍然较差。在我们的系列中，优势狭窄与发生 CC 的风险较高相关。
