Burak Kelly, Angulo Paul, Pasha Tousif M, Egan Kathleen, Petz Jan, Lindor Keith D
Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA.
Am J Gastroenterol. 2004 Mar;99(3):523-6. doi: 10.1111/j.1572-0241.2004.04067.x.
Cholangiocarcinoma (CCA) is a dreaded complication of primary sclerosing cholangitis (PSC); however, marked variability in the incidence of CCA in PSC is reported. Furthermore, limited information exists on risk factors for the development of CCA in PSC. The aim of this study was to determine the incidence of CCA in patients with PSC and to evaluate baseline risk factors for the later development of CCA. From a previous study of the natural history of PSC, we identified 161 patients with PSC who did not have CCA at study entry. Patients were followed until a diagnosis of CCA was established, liver transplantation was performed, or death occurred. Patients were followed for a median of 11.5 yr (interquartile range 4.0-16.1 yr). Fifty-nine patients (36.6%) died, 50 patients (31.1%) underwent liver transplantation, and 11 patients (6.8%) developed CCA. The rate of CCA developing was approximately 0.6% per year. Compared to the incidence rates of CCA in the general population, the relative risk of CCA in PSC was significantly increased (RR = 1,560; 95%CI = 780, 2,793; p < 0.0001). On univariate analysis, a history of variceal bleeding (p < 0.001), proctocolectomy (p= 0.01), and lack of symptoms (p= 0.02) were significant risk factors for CCA with the Mayo Risk Score being marginally significant (p= 0.051). Multivariate analysis determined only variceal bleeding to be a significant risk factor for CCA (RR 24.2; 95%CI: 3.3-67.1). No association was found between the duration of PSC and the incidence of CCA. In conclusion, approximately 7% of PSC patients later developed CCA over a mean follow-up of 11.5 yr, which is dramatically higher than the rates in the general population. Variceal bleeding is a major risk factor for the later development of CCA.
胆管癌（CCA）是原发性硬化性胆管炎（PSC）的一种可怕并发症；然而，据报道PSC中CCA的发病率存在显著差异。此外，关于PSC中CCA发生的危险因素的信息有限。本研究的目的是确定PSC患者中CCA的发病率，并评估CCA后期发生的基线危险因素。从先前一项关于PSC自然史的研究中，我们确定了161例在研究开始时没有CCA的PSC患者。对患者进行随访，直至确诊CCA、进行肝移植或死亡。患者的中位随访时间为11.5年（四分位间距4.0 - 16.1年）。59例患者（36.6%）死亡，50例患者（31.1%）接受了肝移植，11例患者（6.8%）发生了CCA。CCA的年发生率约为0.6%。与一般人群中CCA的发病率相比，PSC中CCA的相对风险显著增加（RR = 1560；95%CI = 780, 2793；p < 0.0001）。单因素分析显示，静脉曲张出血史（p < 0.001）、直肠结肠切除术（p = 0.01）和无症状（p = 0.02）是CCA的显著危险因素，Mayo风险评分具有边缘显著性（p = 0.051）。多因素分析确定只有静脉曲张出血是CCA的显著危险因素（RR 24.2；95%CI：3.3 - 67.1）。未发现PSC病程与CCA发病率之间存在关联。总之，在平均11.5年的随访中，约7%的PSC患者后来发生了CCA，这显著高于一般人群中的发病率。静脉曲张出血是CCA后期发生的主要危险因素。
