Jepsen Peter, Grønbæk Lisbet, Vilstrup Hendrik
Dig Dis. 2015;33 Suppl 2:2-12. doi: 10.1159/000440705. Epub 2015 Dec 7.
The variation that occurs in the incidence patterns of autoimmune liver diseases may provide insight into the risk factors causing the diseases. We systematically reviewed studies on the incidence of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and immunoglobulin G4-associated cholangitis (IAC) in general populations.
We found relevant studies through Medline and Scopus, and we examined whether they were population-based; the way they found cases for inclusion and which diagnostic criteria they used; and whether they used standardization to facilitate comparison with other studies. The 55 identified studies varied greatly in their case-finding methods, and only 14 (25%) of them used a standard population. Reported incidence rates of AIH were around 1 per 100,000 population per year, possibly higher in Scandinavia than in other countries, and a Danish study of the 1994-2012 period found an increasing incidence. A majority of PBC studies found incidence rates of 1-2 per 100,000 population per year and an increasing time trend, but incidence was lower in the Netherlands and New Zealand and higher in North East England. Most studies of PSC found incidence rates around 1 per 100,000 population per year, but there were no incident cases among 100,000 Alaska natives during the period 1984-2000. The incidence of IAC remains unknown.
The incidence of the autoimmune liver diseases is around 1-2 per 100,000 population per year for each disease. The variation in incidence over time and place suggests that there are differences in the prevalence of risk factors for the diseases, but the studies used different methods and so it is difficult to draw firm conclusions. We recommend that groups of investigators conduct multisite studies with identical case-finding methods, and that they use a standard population to account for differences in demographics.
自身免疫性肝病发病率模式的变化可能有助于深入了解引发这些疾病的风险因素。我们系统回顾了关于普通人群中自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）、原发性硬化性胆管炎（PSC）和免疫球蛋白G4相关性胆管炎（IAC）发病率的研究。
我们通过Medline和Scopus检索相关研究，并考察这些研究是否基于人群；病例纳入的查找方式以及所采用的诊断标准；是否采用标准化方法以便与其他研究进行比较。所确定的55项研究在病例查找方法上差异很大，其中只有14项（25%）采用了标准人群。报道的AIH发病率约为每年每10万人1例，在斯堪的纳维亚地区可能高于其他国家，一项丹麦对1994 - 2012年期间的研究发现发病率呈上升趋势。大多数PBC研究发现发病率为每年每10万人1 - 2例且呈上升趋势，但在荷兰和新西兰发病率较低，在英格兰东北部较高。大多数PSC研究发现发病率约为每年每10万人1例，但在1984 - 2000年期间，10万阿拉斯加原住民中未出现新发病例。IAC的发病率仍然未知。
每种自身免疫性肝病的发病率约为每年每10万人1 - 2例。发病率随时间和地点的变化表明这些疾病的风险因素患病率存在差异，但研究采用了不同方法，因此难以得出确凿结论。我们建议研究团队采用相同的病例查找方法进行多中心研究，并使用标准人群来考虑人口统计学差异。
