Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community
Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community
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DOI：
10.1016/j.gastro.2003.07.011
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发表时间：
2003-11-01
期刊：
影响因子：
29.4
通讯作者：
Melton, LJ
中科院分区：
文献类型：
--
作者：
Bambha, K;Kim, WR;Melton, LJ
背景与目的：原发性硬化性胆管炎（PSC）在美国的流行病学尚不清楚。我们报告了明尼苏达州奥姆斯特德县PSC的发病率、临床谱和预后。方法：利用明尼苏达州奥姆斯特德县的医疗记录链接系统罗切斯特流行病学项目，我们确定了患有PSC的县居民，并根据临床、生化、放射学和组织学标准确诊。结果：1976-2000年共有22例PSC符合诊断标准。年龄调整后（至2000年美国白人）男性PSC发病率为：1.25 / 10万人年（95% CI, 0.70 - 2.06），而女性为6.54 / 10万人年（95% CI, 0.22 - 1.12）。2000年PSC的患病率为20.9 / 10万男性（95% CI, 9.5 - 32.4），而只有6.3 / 10万女性（95% CI, 0.1 - 12.5）。73%的病例患有炎症性肠病，其中大多数患有溃疡性结肠炎。在明尼苏达州年龄和性别相似的白人人群中，PSC患者的生存率明显低于预期（P < 0.001）。结论：这些数据代表了美国PSC发病率和患病率的第一个基于人群的估计。在同一人群中，PSC的发病率和流行率约为先前描述的原发性胆汁性肝硬化的三分之一。我们的数据表明，PSC在美国的患病率及其伴随的医疗负担明显高于先前的估计。
Background & Aims: The epidemiology of primary sclerosing cholangitis (PSC) in the United States is unknown. We report the incidence, clinical spectrum, and outcomes of PSC in Olmsted County, Minnesota. Methods: Using the Rochester Epidemiology Project, a medical records linkage system in Olmsted County, Minnesota, we identified county residents with PSC, and the diagnosis was confirmed according to clinical, biochemical, radiographic, and histologic criteria. Results: Twenty-two patients met diagnostic criteria for PSC in 1976-2000. The age-adjusted (to 2000 U.S. whites) incidence of PSC in men was :1.25 per 100,000 person-years (95% CI, 0.70 to 2.06) compared with 6.54 per 100,000 person-years (95% CI, 0.22 to 1.12) in women. The prevalence of PSC in 2000 was 20.9 per 100,000 men (95% CI, 9.5 to 32.4) and only 6.3 per 100,000 women (95% CI, 0.1 to 12.5). Seventy-three percent of cases had inflammatory bowel disease, the majority with ulcerative colitis. Survival among PSC patients was significantly less than expected for the Minnesota white population of similar age and gender (P < 0.001). Conclusions: These data represent the first population-based estimates of the incidence and prevalence of PSC in the United States. The incidence and prevalence of PSC were approximately one third of those previously described for primary biliary cirrhosis in the same population. Our data suggest that the prevalence of PSC in the United States, with its attendant medical burdens, is significantly greater than-previously estimated.