Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Utah, Salt Lake City, UT.
Hepatology. 2013 Oct;58(4):1392-400. doi: 10.1002/hep.26454. Epub 2013 Aug 13.
The epidemiology and natural history of pediatric primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC), and autoimmune hepatitis (AIH) are not well characterized. Using multiple, overlapping search strategies followed by a detailed records review, we identified all cases of pediatric PSC, ASC, AIH, and inflammatory bowel disease (IBD) in a geographically isolated region of the United States. We identified 607 cases of IBD, 29 cases of PSC, 12 cases of ASC, and 44 cases of AIH. The mean age at diagnosis was 13.0 years for PSC, 11.3 years for ASC, and 9.8 years for AIH. The incidence and prevalence of PSC, ASC, and AIH were 0.2 and 1.5 cases, 0.1 and 0.6 cases, and 0.4 and 3.0 cases per 100,000 children, respectively. The mean duration of follow-up was 5.9 years. The probability of developing complicated liver disease within 5 years of the diagnosis of liver disease was 37% [95% confidence interval (CI) = 21%-58%] for PSC, 25% (95% CI = 7%-70%) for ASC, and 15% (95% CI = 7%-33%) for AIH. The 5-year survival rates with the native liver were 78% (95% CI = 54%-91%) for PSC, 90% (95% CI = 47%-99%) for ASC, and 87% (95% CI = 71%-95%) for AIH. Cholangiocarcinoma developed in 2 of the 29 PSC patients (6.9%). PSC occurred in 9.9% of patients with ulcerative colitis (UC) and in 0.6% of patients with Crohn's disease (CD). ASC occurred in 2.3% of UC patients and 0.9% of CD patients. AIH occurred in 0.4% of UC patients and in 0.3% of CD patients. Liver disease occurred in 39 of 607 IBD patients (6.4%) overall.
Immune-mediated liver diseases are important sources of morbidity in children. Using a population-based design, this study quantifies the burden and natural history of immune-mediated liver disease in children.
描述儿科原发性硬化性胆管炎（PSC）、自身免疫性硬化性胆管炎（ASC）和自身免疫性肝炎（AIH）的流行病学和自然史。
使用多种重叠的搜索策略，然后进行详细的记录审查，我们确定了美国一个地理位置孤立的地区所有儿科 PSC、ASC、AIH 和炎症性肠病（IBD）的病例。我们确定了 607 例 IBD、29 例 PSC、12 例 ASC 和 44 例 AIH。PSC 的平均诊断年龄为 13.0 岁，ASC 为 11.3 岁，AIH 为 9.8 岁。PSC、ASC 和 AIH 的发病率和患病率分别为 0.2 和 1.5 例、0.1 和 0.6 例、0.4 和 3.0 例/10 万儿童。平均随访时间为 5.9 年。PSC 患儿确诊后 5 年内发生复杂肝病的概率为 37%（95%CI：21%-58%），ASC 为 25%（95%CI：7%-70%），AIH 为 15%（95%CI：7%-33%）。PSC 患儿的 5 年生存率为 78%（95%CI：54%-91%），ASC 为 90%（95%CI：47%-99%），AIH 为 87%（95%CI：71%-95%）。PSC 患者中有 2 例发生胆管癌（6.9%）。溃疡性结肠炎（UC）患者中 PSC 发生率为 9.9%，克罗恩病（CD）患者为 0.6%。UC 患者中 ASC 发生率为 2.3%，CD 患者为 0.9%。UC 患者中 AIH 发生率为 0.4%，CD 患者为 0.3%。总体而言，607 例 IBD 患者中有 39 例（6.4%）发生肝病。
免疫介导的肝病是儿童发病的重要原因。本研究采用基于人群的设计，量化了儿童免疫介导性肝病的负担和自然史。
