Cholangiocarcinoma in primary sclerosing cholangitis:: Risk factors and clinical presentation
Cholangiocarcinoma in primary sclerosing cholangitis:: Risk factors and clinical presentation
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DOI：
10.1080/003655202760373434
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发表时间：
2002-10-01
影响因子：
1.9
通讯作者：
Schrumpf, E
中科院分区：
文献类型：
--
作者：
Boberg, KM;Bergquist, A;Schrumpf, E
背景：原发性硬化性胆管炎（PSC）是发生胆管癌（CC）的高危因素。由于可能选择有CC风险的患者进行早期肝移植，因此识别任何诱发因素是一项挑战。我们比较了大量PSC患者的临床表现和自然病史，并与后来的CC发展，以确定与CC风险相关的特征。方法：从来自五个欧洲国家的394例PSC患者中收集了临床和实验室数据。该队列包括48例（12.2%）CC患者。结果：24例（50%）患者在PSC诊断后第一年内诊断为CC，13例（27%）患者在预期肝移植时诊断为CC。在发生CC的组中，在诊断PSC时黄疸、瘙痒、腹痛和疲劳明显更常见，但排除第一年内诊断的病例后并非如此。炎症性肠病在PSC前至少1年被诊断，在CC发展的患者中比没有CC发展的患者更常见（分别为90%和65%; P = 0.001）。在诊断PSC之前，发生CC的患者的炎症性肠病持续时间显著长于其余组（分别为17.4年和9.0年;多变量分析中P = 0.009）。结论：高比例的CC病例在诊断PSC后的第一年内被诊断出来。长期的炎症性肠病史是CC发展的危险因素。
Background: Primary sclerosing cholangitis (PSC) confers a high risk of cholangiocarcinoma (CC) development. Since patients at risk of CC may be selected for early liver transplantation, it is a challenge to identify any predisposing factors. We compared the presentation and natural history of a large number of PSC patients with and without later CC development to identify features associated with risk of CC. Methods: Clinical and laboratory data from presentation and follow-up were collected from 394 PSC patients from five European countries. The cohort included 48 (12.2%) patients with CC. Results: CC was diagnosed within the first year after diagnosis of PSC in 24 (50%) cases and in 13 (27%) patients at intended liver transplantation. Jaundice, pruritus, abdominal pain and fatigue were significantly more frequent at diagnosis of PSC in the group that developed CC, but not after exclusion of cases diagnosed within the first year. Inflammatory bowel disease was diagnosed at least 1 year before PSC more often among patients with CC development than among those without (90% and 65%, respectively; P = 0.001). The duration of inflammatory bowel disease before diagnosis of PSC was significantly longer in patients who developed CC than in the remaining group (17.4 years and 9.0 years, respectively; P = 0.009 in multivariate analysis). Conclusions: A high proportion of CC cases is diagnosed within the first year after diagnosis of PSC. A long history of inflammatory bowel disease is a risk factor for CC development.